Medical Quiz Flashcards

Question

TNF inhibitors pros and cons And list

Answer 1

Pros: - Good for psoriatic arthritis (both peripheral and axial) - Pregnancy use (certrolizumab) Cons: - Weight gain - Avoid in Hep B - Avoid in demyelinating disease - Production of antibodies/resistance - Avoid in CCF class 3 or 4

Answer 2

Psoriatic nail disease Flexural psoriasis, scalp psoriasis, post auricular psoriasis Obesity Smoker Elevated CRP at baseline

Answer 3

Younger age at onset More extensive disease Rapid progression Hair loss of greater than one-year duration Nail dystrophy Ophiasis pattern Family history of alopecia areata Presence of atopy or other autoimmune diseases

Answer 4

TCS Dapsone PO steroid Acitretin Colchicine nbUVB CsA TNF-alpha

Answer 5

Nail bed: Onycholysis Splinter haemorrhage Oil drop/spot Subungual hyperkeratosis Nail matrix: Pitting Trachyonychia Ridging Leukonychia

Answer 6

Admission Bed rest Bland emollients Fluid and protein replacement Thermoregulation Remove triggers - Stop tar or dithranol (can be done abruptly), wean topical corticosteroids - Treat infection Weak topical corticosteroids in subacute forms Systemic therapies - Acitretin first line 0.5mg/kg/day - PUVA - MTX - Cyclosporine - Dapsone 50-200mg/day: especially atypical variants and children - Oral corticosteroids should only be used when urgent control of metabolic complications is needed (serious relapses may occur with reduction of therapy unless another therapy such as acitretin or MTX is given simultaneously) - TNF alpha inhibitors - New: spesolimab IL36

Answer 7

IgA paraproteinaemia Multiple myeloma Lymphoma RA SLE

Answer 8

subscores for all 3 of erythema, thickness and scaling, to slight or better, or sustained at this level, as compared to the baseline values OR reduction by 75% or more in the skin area affected, or sustained at this level, as compared to the baseline value for this treatment cycle

Answer 9

Preg cat: C Adalimumab: For PsO: 80mg wk 0, 40mg wk 1, 40mg every 2 weeks For HS: 160mg wk 0, 80mg wk 2, 40mg wk 4 and then weekly Etanercept: 50mg subcut weekly Infliximab: 5mg/kg wk 0, 2, 6 then 8wkly CIs: active infection, MS, malignancy, CCF class 3 or 4

Answer 10

SUP DEVIL SCCs URTI/infections Urticaria Psoriasis Perforating dermatoses DM Eczema EN Vasculitis ISRs Lupus CCF Weight gain

Answer 11

Preg cat B1 Ustekinumab (stelara) IL12/23 (p40 subunit) /=101kg 90mg wk 0, 4, then 12 wkly Other IL23s (all p19 subunit) Guselkumab (tremfya): 100mg wks 0, 4, then 8wkly Tildrakizumab (ilumya): 100mg wks 0, 4 then 12wkly Risankizumab (skyrizi): 150mg wks 0, 4 then 12wkly CIs: active infection, TB, hep B malignancy MS severe CHF

Answer 12

Preg cat C Ixekizumab (Taltz) IL17A 160mg wk 0, then 80mg 2wkly for 12 wks, then 4wkly Secukinumab (Cosentyx) IL17A 300mg wkly for 5 weeks, then monthly Bimekizumab (Bimzelx) IL17A and IL17F 320mg 4wkly for 5 doses, the 8 wkly CIs: IBD, infections, live vaccines

Answer 13

Rifampicin 600mg monthly Dapsone 100mg daily If 1+ bacilli then also clofazamine 300mg monthly and 50mg daily For 1 year 6 months if no bacilli (and no clofazamine)

Answer 14

RIPE 600/300/30/15 Rifampicin 600mg/day Isoniazid 300mg/day Pyrazinamide 30mg/kg/day Ethambutol 15mg/kg/day 2 months of the 4 Then extra 4 months of rifampicin and isoniazid (6 months total)

Answer 15

MMR VZV Rotavirus Yellow fever Typhoid BCG

Answer 16

Phenytoin Liver - acitretin, ETOH / Loop diuretics Aspirin, acitretin, alcohol NSAIDs (Tetracycline) Sulfur drugs - bactrim, dapsone

Answer 17

HAS LIGHT Headache, dizzy, fatigue, mood changes Alopecia SCC Lung: acute pneumonitis, interstitial pulmonary fibrosis Infections GI: oral stomatitis, N+V, cramps, diarhhoea, LFTs, cirrhosis Haem: macrocytosis, cytopaenias TEN/drug eruptions, photosensitivity, recall, acral erythema

Answer 18

Atopy thyroid vitiligo IBD T1DM

Answer 19

Acute systemic sclerosis (rapidly progressive, diffuse) Renal crisis, renal involvement Breast cancer

Answer 20

Severe systemic sclerosis

Answer 21

Systemic sclerosis (or mixed or Sjogrens)

Answer 22

A CHIMP Anti TNFs Chlopromazine Hydralazine Isoniazid Minocycline Methyldopa Phenytoin

Answer 23

PIC MII CLINT PMLE Infusion reactions Cytopaenias Malignancy, MSK pain Infection - Hep B, TB, URTI, UTI Immunodeficiency (acquired) - low immunoglobulins Cardiac - arrythmias, heart failure, angina, MI Lungs - pneumonitis Itch Nausea, vomiting abdo pain diarrhoea TEN/SJS

Answer 24

AH FIT Anaphylaxis (IgA def) Hypersensitivity - EM, dermatitis, purpura Fluid overload Infusion reaction - headache, fever, chills, nausea, vomiting, chest pain, tachycardia, blood pressure changes, wheezing – generally mild, slow infusion Thromboembolic event

Answer 25

MISH GRG Malignancy: bladder cancer, SCC, lymphoma/leukaemia Infection Skin: Anagen effluvium, pigmentation skin/nails, pigmented band on teeth, urticaria, SJS Haem: cytopenias GI: N,V,D, hepatotoxicity Reproductive – ovarian failure, azoospermia (Condoms for 4 months after treatment) GU: bladder fibrosis, hemorrhagic cystitis, bladder cancer

Answer 26

Fever, malaise, arthralgia, myalgia Ocular - conjunctivitis, episcleritis Lungs - neutrophilic alveolitis Multifocal sterile osteomyelitis Renal - GN GI - colitis, hepatitis, pancreatitis Aseptic meningitis, encephalitis

Answer 27

Need both major and 2 minor Major criteria: 1. Abrupt onset of typical cutaneous lesions 2. Histopath consistent Minor criteria: 1. Preceded by infection, vaccination, associated malignancy or inflammatory disorder or drug exposure or pregnancy 2. Presence of fever and constitutional signs and symptoms 3. Leukocytosis Excellent response to systemic corticosteroids FLAT Fever Leukocytosis Associations Treatment response

Answer 28

Dermal oedema Diffuse nodular and perivascular neutrophilic infiltrate No vasculitis Leukocytoclasia Endothelial cell oedema, red blood cell extravasation, but no fibrinoid necrosis

Answer 29

Investigate for and treat any associations Potent TCS, ILCS Pred 0.5-1mg/kg/day for 2-6 weeks Colchicine Dapsone Potassium iodide NSAIDs "If malignancy and infection have been excluded, then..." CsA, MTX TNFs Anakinra Rituximab IVIG Thalidomide

Answer 30

Clinical: - pustules - vegetating plaques - crusting - often flexural (axillae, groin) and scalp - asymmetrical - oral: erythema, pustules, erosions, 'snail-trail' - vulva, nose, eyelids Associations: - IBD, UC > Crohns - Bacterial infections - HIV, ETOH, T cell lymphoma, CML Histo: - Pseudoepitheliomatous hyperplasia - Intraepidermal and subepidermal neutrophils, eosinophilic and neutrophilic microabscesses/subcorneal abscesses - Mixed dermal inflammatory infiltrate - Negative DIF (ddx pemphigus) Also note peripheral eosinophilia Treatment: - TOP CS, CNI - Abx (tetracyclines, augmentin) - PO pred - AZA - Dapsone - CsA - MTX - Infliximab and other TNFs - Zinc supplements

Answer 31

DQ SAME PLACES Dapsone Quinine Sulfonamides (bactrim, SSZ) Aspirin, NSAIDs Antibiotics ACE inhibitors Mino/tetracyclines Metronidazole Epileptics Paracetamol Laxatives Azoles Congestants (pseudoephedrine) Calcium channel blockers Erythromycin Sedatives

Answer 32

FRANDS Frusemide, thiazide Retinoids Antibiotics (tetracyclines) Amiodarone Aspirin NSAIDs (esp naproxen) Dapsone Dialysis Sulfonamides Sunbeds

Answer 33

BAD FACE Bactrim Antibiotics, beta lactams, macrolides, metronidazole Diltiazem Frusemide Allopurinol, antimalarials Cimetidine Epileptics Terbinafine

Answer 34

Plaque Linear Bullous/pansclerotic Deep Keloidal Guttate Nodular Atrophoderma or pasini and pierini

Answer 35

TOP CS potent PUVA/UVA1 PO pred MTX Penicillamine Isotretinoin Physiotherapy Surgery (revision, reconstruction) New: abatacept, tocilizumab, ruxolitinib

Answer 36

Acrokeratosis neoplastica

Answer 37

Spongiosis and lichenoid pattern Lymphocytic infiltrate perivascular and perieccrine

Answer 38

Acanthosis Spongiosis Lymphocytic exocytosis CD8 T lymphocytes

Answer 39

Acrodermatitis chronica atrophicans

Answer 40

Clinical: Bullous (aw AML) Pustular (aw IBD) Subcutaneous/neutrophilic panniculitis Facial erysipelas like Neutrophilic dermatosis of the dorsal hands Histological: Histiocytoid (stain MPO - myeloperoxidase) Lymphocytic Eosinophilic

Answer 41

MINTS DRIPS Malignancy (15-30%) - haem, AML Infection (~25%) (viral, bacterial, mycobacterial, fungi) - URTI, CMV, EBV, Hep, HIV, Strep, Dimorphic fungi Neutrophilic dermatoses, other Thyroid, other autoimmune Sarcoid Drugs (~10%) RA IBD Pregnancy SLE and CTD

Answer 42

Granulocyte growth factors All-trans-retinoic acid Antibiotics (bactrim, minocyclin, quinolones) Abacavir AZA Frusemide Hydralazine NSAIDs Interferon alpha Imatinib Checkpoint inhibitors ----------------------------------------- Allan's Lollies All-trans-retinoic acid Antibiotics - bactrim, minocycline, quinolones Frusemide (fruit flavoured) Gummy - granulocyte growth factors Toothache - NSAIDs 'check for cavities' Abacavir Checkpoint inhibitors Sugar high - mad as a hatter Imatinib Azathioprine Hydralazine Interferon alpha

Answer 43

Vacuoles E1 enzyme X-linked Autoinflammatory Somatic - acquired somatic mutation in UBA1, encodes the major E1 enzyme - neutrophilic dermatoses, polychondritis, and vasculitis (livedo racemosa, giant cell arteritis, polyarteritis nodosa) - Inflammatory arthritis - Haem: anaemia (macrocytic with normal folate and vit B12), thrombocytopaenia, myelodysplastic syndrome, multiple myeloma, MGUS - Neutrophilic alveolar infiltrate - Complications: Resp failure Progressive anaemia Multiorgan involvement (kidney, heart, brain, bowel) Thrombotic events (venous thromboembolism, arterial stroke)

Answer 44

Biopsy FBE (+film), UEC, LFT ESR, CRP beta HCG For associations, depending on symptoms: TFTs ACE Hep B, C, HIV ASOT and anti DNase B Swab throat Stool culture Malignancy screen incl LDH, SPEP Faecal calprotectin +/- scopes RF ANA ENA dsDNA C3 C4 Swabs bacterial viral Pre-treatment: G6PD, HbA1c + preimmunosuppression screen

Answer 45

Admit if needed Treat underlying cause Consider comorbidities - pain, depression, PVD Consider syndromes Goals - halt inflammation, heal wound Approaches - wound care, local therapy, systemic therapy Top CS ILKA Top Tacro Oral abx Pred 0.5-1mg/kg/day, wean over months (therefore Ca, Vit D) Methylpred 1g daily for 3-5 days TNF alpha (adalimumab, infliximab) Ustekinumab Dapsone Colchicine KI IVIG CsA, AZA (50-100mg BD), MTX, MMF IL1 e.g. anakinra, canakinumab Other: thalidomide, cyclophosphamide, plasmapheresis IL23

Answer 46

IBD 20-30% RA 20% Haem disease AML>CML, MDS 15-25% Monoclonal gammopathies 15% PAPA (pyogenic arthritis, PG, acne: PSTPIP1 mutation) PASH (PG, acne, HS) PAPASH (pyogenic arthritis, PG, acne, HS) Drugs Other: dermatomyositis, other neutrophilic derm, immunodeficiency syndromes

Answer 47

Ulcerative Peristomal Pustular Bullous Granulomatous superficial Pyodermatitis pyostomatitis vegetans Extracutaneous (lungs most common, other: bone, liver, pancreas, spleen, kidney, CNS)

Answer 48

PIGI: Propylthiouracil, carbimazole Isotretinoin GCSF Interferon

Answer 49

Biopsy, culture, DIF FBE + film, UEC, LFT, ESR, CRP ANA, ENA, dsDNA, C3, C4 ANCA APLA RF SPEP, flow cytometry Faecal calprotectin, scopes FOBT, stool OCP Malignancy screening CXR (extracutaneous) Urinalysis USS legs doppler arterial and venous (wound healing)

Answer 50

NICE PLANT Neutrophilic Infective Calciphylaxis Enzymatic - pancreatic PAN Lymphoma Alpha 1 antitrypsin NLD Trauma

Answer 51

Both major + 2 minor Major: 1. Rapid progression of painful, necrolytic cutaneous ulcer with irregular, violaceous, undermined border 2. Other cause of cutaneous ulcer excluded Minor: 1. Pathergy or cribriform scarring 2. Systemic disease associated with PG 3. Histopath consistent (sterile dermal neutrophilia +/- mixed inflammation +/- lymphocytic vasculitis) 4. Treatment response (rapid response to steroids) PATH Pathergy Associations Treatment Histo

Answer 52

Cutaneous: - Cutaneous ulceration, PG-like - Sweets-like lesions - EN-like lesions - Pathergy - Pustular vasculitic lesions - Acral and facial sterile vesiculopustules - Palpable purpura Extracutaneous EYE CARVING: - Eye - uveitis (posterior is most characteristic finding), secondary glaucoma, cataracts, conjunctivitis, keratitis, scleritis, vitreous haemorrhage, optic neuritis, retinal vasculitis - Cardiopulmonary - coronary arteritis, valvular disease, myocarditis, recurrent ventricular arrhythmias, pulmonary artery aneurysms - Arthritis - Renal (GN) - Vascular - aneurysmal or occlusive arterial disease, s/f or DVT - Neurologic - Acute meningo-encephalitis, CN palsies, brainstem lesions - GI - abdo pain, haemorrhage, ulcerations

Answer 53

Viral and bacterial swabs - excl HSV MDT - ophthalm, rheum, gastro, cardiology, resp, renal Biopsy - angiocentric neutrophilic infiltrate wth leukocytoclastic or lymphocytic infiltrate +/- thrombosis HLAB51 Syphilis and STI screen ECG, TTE Imaging guided by rheum FBE, UEC, LFTs, ESR, CRP Urine (GN) Pre-treatment bloods

Answer 54

Mouth and genital ulcers and inflamed cartilage (Behcet's + relapsing polychondritis)

Answer 55

Admit as needed MDT Oral/genital/eye care - incl inhaled CS, anaesthetics General measures: quit smoking, mild diet TCS + ILCS PO pred 0.5-1mg/kg Colchicine Dapsone Apremilast AZA CsA MMF MTX TNFs Interferon alpha 2 IVIG Thalidomide IL1

Answer 56

Revised criteria - need 4 or more points Ocular lesions (recurrent) - 2 points Oral aphthosis (recurrent) - 2 Genital aphthosis (recurrent) - 2 Skin lesions (recurrent) - 1 CNS lesions - 1 Vascular manifestations - 1 Positive pathergy test - 1 (PS CV)

Answer 57

Bowel associated dermatosis arthritis syndrome - Post bowel surgery - Diarrhoea, malabsorption - Arthritis (episodic, polyarthritis, asymmetrical) - Erythematous macules and pustules, favour proximal extremities, may occur at 4-6 weekly intervals - Nodular panniculitis/EN - Serum sickness like constitutional flu like Sx - fever, chills, malaise, myalgias - Histo: neutrophilic infiltrate dermis

Answer 58

Electrolyte imbalance from diarrhoea Hepatic dysfunction and failure Calcium oxalate renal calculi Gallstones Zn deficiency Vit A deficiency Thiamine (B1) deficiency - beriberi Hyperuricaemia

Answer 59

Surgical revision or resection Pred (not curative) Abx for 4-8 weeks - doxy, mino, clinda, metronidazole Colchicine, dapsone CsA, AZA, MMF TNF

Answer 60

Synovitis Acne Pustulosis Hyperostosis and Osteitis Pustular dermatosis + aseptic osteoarticular lesions Skin: - acne, follicular occlusion tetrad - palmoplantar pustulosis - pustular psoriasis - sweets, PG, Behcets - subcorneal pustular dermatosis - LABD Also a/w IBD

Answer 61

Biopsy Bone scintigraphy (increased uptake) MRI FBE, UEC, LFT - leukocytosis ESR, CRP - elevated

Answer 62

NSAIDs Pred AZA MTX TNF IL1 Bisphosphonates for bones (isotretinoin can treat acne but flare SAPHO)

Answer 63

Associations: - AML - ChemoTx - cytarabine - benign self-limited childhood variant (= idiopathic plantar hidradenitis) Histo: - Neutrophils infiltrating eccrine gland, epithelial necrosis Treatment: - Exclude infection - PO pred - Dapsone, colchicine - NSAIDs

Answer 64

Posterior fossa and other structural brain malformations Haemangioma Arterial anomalies of cervical and cerebral vessels Cardiac defects (especially Coarctation of the aorta) Eye anomalies Sternal defects and Supraumbilical raphe

Answer 65

Urgent referral to neurology for MRI/A head and neck - ideally before 12 weeks of age (can avoid GA) Urgent referral to cardiology (echo) Urgent referral to ophthalmology Consider TFTs and LFTs

Answer 66

Markers of laryngeal haemangiomatosis Airway haemangiomas - Noisy breathing - Biphasic stridor Refer to ENT

Answer 67

Spinal dysraphism, increased risk if: - >2.5cm - Ulcerated - Involuted - Additional signs: deviated gluteal cleft, lipomas, skin appendage

Answer 68

Lower body/lumbosacral hemangioma and lipomas or other cutaneous anomalies (e.g. “skin tags”) Urogenital anomalies (bladder, ureters, gentalia) and ulceration of the hemangioma Myelopathy (spinal dysraphism) Bony deformities Anorectal and arterial anomalies Renal anomalies

Answer 69

MDT - urology, gynae, neurosurg, paeds USS lower abdo pelvis - kidneys, urinary tract, genitalia USS spine, ideally <4 months - for spinal dysraphism - if indeterminate, refer to neurosurg for MRI spine

Answer 70

Visceral haemangiomatosis Consumptive hypothyroidism Investigate if >/= 5 Liver USS TFTs

Answer 71

Lobular Very cellular within lobules Uniform vessel morphology Outer layer of pericytes (spindle shaped) Inner layer of endothelial cells Containing typical microtubular Weibel-Palade bodies GLUT1 positive (also +ve in angiosarcoma and angiokeratoma) Lewis Y

Answer 72

Infantile haemangioma with minimal or arrested growth Often present at birth Often segmental pattern Telangiectasia Venules Matte erythema Alternating light and dark areas Peripheral pallor Peripheral bright papules Ulceration Reticular High risk of extracutaneous associations (PHACES, LUMBAR) Higher risk ulceration

Answer 73

Hypoglycaemia (FTT, poor feeding, gastroenteritis) Cardiovascular disease (heart block 2nd or 3rd degree, persistent bradycardia, coarctation of aorta Wheezing/bronchospasm ?Intracranial arterial anomalies (PHACES)

Answer 74

DG PANTS Doxorubicin Griseofulvin PPI ANti-TNFs, Anti-HTN, Anti-epileptics Terbinafine, thiazides Statins

Answer 75

DIG Dapsone Isoniazid Griseofulvin

Answer 76

ACLE >90% SCLE 30-50% DLE 10-20%

Answer 77

TREX1 or SAMHD1

Answer 78

AR Sterile fevers, progressive developmental delay, chilblains

Answer 79

Mother has SSA/Ro autoantibodies Screen for: - Congenital heart block (ECG/echo) - Cytopaenias (FBE) - Hepatobiliary disease (LFTs) Repeat every 2-3 months if normal Monitor growth and head circumference Counsel: - No scarring, but dyspigmentation may persist for months, some residual telangiectasias perhaps - Risk NLE in subsequent pregnancies - consider HCQ in subsequent pregnancies - Risk of mother and child developing AI-CTD

Answer 80

Livedo reticularis Raynaud's Palmar erythema Erythromelalgia Nail fold telangiectasia Ulceration Alopecia Papulonodular mucinosis Anetoderma

Answer 81

Sunprotection - Exacerbation skin, skin cancer risk, exacerbation of SLE Smoking cessation Topical and intralesional CS TOP CNI HCQ - <5mg/kg/day - May take 2-3 months to notice improvement Systemic corticosteroids Oral retinoids Thalidomide Lenalidomide Dapsone (mostly just for bullous) MMF, AZA, MTX Sulfasalazine

Answer 82

MONARCHS MSK - joint involvement Oral/cutaneous - non-scarring alopecia, oral ulcers, SCLE, DLE, ACLE Neuropsychiatric - delirium, psychosis, seizure ANA +ve, APLS, low C3/4, anti dsDNA or anti-Smith Renal - proteinuria or lupus nephritis on renal biopsy Constitutional (fever >38) Haematologic - leukopaenia, thrombocytopaenia, acute pericarditis Serosal - pleural or pericardial effusion, acute pericarditis

Answer 83

DIF - granular deposition of IgG, IgM, occasional IgA and complement at DEJ and around hair follicles ACLE Vacuolar alteration of basal layer Apoptotic keratinocytes Epidermal atrophy Lymphocytic infiltrate in upper dermis Dermal oedema Dermal mucin Focal haemorrhage SCLE Vacuolar alteration of basal layer Apoptotic keratinocytes Epidermal atrophy Lymphocytic infiltrate in upper dermis Dermal mucin DLE Vacuolar alteration of basal layer Apoptotic keratinocytes Hyperkeratosis Pilosebaceous atrophy Follicular plugging Lymphocytic infiltrate in upper dermis Lymphocytic infiltrate in lower dermis Periadnexal lymphocytic infiltrate Dermal mucin Tumid lupus Lymphocytic infiltrate in upper dermis Lymphocytic infiltrate in lower dermis Dermal mucin

Answer 84

Terbinafine Phenytoin PPI NSAIDs TNFs Penicillamine Statin Hydroxyurea

Answer 85

Antisynthetase antibodies (anti Jo1, PL7) Fever Erosive polyarthritis Mechanics hands Gottron papules Raynaud phenomenon ILD

Answer 86

Classic cutaneous disease Milder muscle disease Not a/w malignancy or ILD Benign prognosis Most responsive to treatment

Answer 87

Amyopathic More severe/rapidly progressive ILD and high mortality Digital and mouth ulcers Diffuse alopecia Painful palmar papules Panniculitis Arthritis Cutaneous ulcerations - periungual region and overlying Gottron papules and sign Inverse Gottron papules

Answer 88

Malignancy association (37.5%) Calcinosis cutis More severe myopathy/contractures, myalgia GI bleeding secondary to vasculitis/vasculopathy Peripheral oedema Cutaneous ulceration Dysphagia Milder skin disease

Answer 89

High malignancy risk (20-65%) Hypomyopathic Lipodystrophy in children Ovoid palatal patch Psoriasis like patches and plaques Severe photosensitive cutaneous disease Atrophic hypopigmented patches/red-on-white telangiectatic patches Palmar hyperkeratosis GI involvement

Answer 90

Angel wing sign Highest risk HCQ drug eruption May present with clinically amyopathic DM

Answer 91

Sudden severe progressive muscle weakness Cardiac involvement Dysphagia Treatment resistance

Answer 92

Pulmonary - ILD (esp MDA5) - Pulm HTN - Serositis Cardiac - Myocarditis - Myocardial fibrosis - Arrhythmias - CCF GI - Dysphagia - Dysmotility of gastric + small intestine - GI infarction/perforation Vascular - Cutaneous vasculopathy - Vasculitis (more in juvenile), also CNS and retinal vasculitis Endocrine in juvenile - Lipodystrophy - Growth failure

Answer 93

FSE + LNs Neuro/strength exam FBE, UEC, LFTs CK LDH Myositis panel ANA, ENA, dsDNA, C3/4 Skin/muscle bx MRI muscles PFTs CXR, CT CAP ECG, echo Barium swallow Age-appropriate or symptom directed malignancy screen - transvaginal USS in women, mammogram, cervical screening, scopes, FOBT, PSA in men, CA-125 CA19-9, urinalysis Annual malignancy screening for 3 years DEXA if long term pred BSL, HbA1c

Answer 94

For cutaneous: Photoprotection Topical CS, CNI HCQ Retinoids For cutaneous/systemic: Oral prednisolone - 1mg/kg/day tapered to 50% over 6 months and to zero over 2-3 years MTX (not if ILD) AZA MMF CsA IVIG Rituximab Anti TNF cyclophosphamide JAKi Physio, exercise, rehab for muscles

Answer 95

General measures - Avoid trauma - Good skin care - Keep hands warm (esp if Raynauds) - Analgesia Topicals - CS - Sodium thiosulfate (also IL) - Aldara Orals ABCCC IWARP - Aluminium hydroxide - Bisphosphonates - Colchicine - Calcium channel blockers - diltiazem - Cinacalcet - IVIG - Warfarin - Abatacept - Rituximab - Probenacid Surgical - curette, excision Ablative laser - CO2 Extracorporeal shockwave lithotripsy

Answer 96

Genitourinary, ovarian, colon, nasopharyngeal (Also breast, lung, gastric, pancreatic, lymphomas)

Answer 97

- Peripheral/marginal curvilinear (crown) vessels, do not cross midline - Central large homogenous pink or yellow-orange globules - Erythema

Answer 98

- Epidermal hyperplasia - Hypergranulosis - Cup-shaped or invaginated or crater - Enlarged keratinocytes - Intracytoplasmic eosinophilic inclusion bodies, homogenous pink-purple globules = molluscum bodies - Downward lobulated growth of epidermis - +/- inflammatory response - Giemsa stain

Answer 99

- No treatment, spontaneous clearance average 6-9 months, may persist for 3-4 years, only 2 months if solitary - If adult, investigate for other STIs/HIV - Physical/surgical: manual extraction, tape stripping, cryotherapy, curettage, electrodessication, laser (PDL>CO2) - Cantharadin 0.7% (2-4 weekly) - Imiquimod 5% (3-5x per week for 6-16 weeks) - Benzoyl peroxide 10% - TOP retinoid - Sal acid 20% - Podophylotoxin 0.5% - TCA 20-35% If HIV - HAART, cidofovir, interferon alpha

Answer 100

- Trichodysplasia spinulosa associated polyomavirus - Solid organ Tx with immunosuppression or leukaemia, lymphoma with ChemoTx - Can get leonine facies and alopecia brows and lashes - Rx: reduce immunosuppression, TOP cidofovir, oral valganciclovir

Answer 101

CRASH and burn Fever >39 at least 5 days PLUS 4 of the following: Conjunctival erythema/conjunctivitis Rash Adenopathy Strawberry tongue or other mucosal change Hands and feet - oedema, pain, desquamation

Answer 102

IVIG 2g/kg over 12 hours Aspirin (if need to escalate - CS and infliximab)

Answer 103

Coronary artery aneurysm or ectasia Myocarditis Pericardial effusion CHF Valvular disease Macrophage activation syndrome Arthritis CNS - aseptic meningitis Otitis media GI - pancreatitis, hepatitis GU - urethritis Facial nerve palsies Anterior uveitis

Answer 104

Papular acrodermatitis of childhood EBV, Hep B (not so much in Aus), enteroviruses usually in Aus, many viruses, bacteria, vaccines Extremities, extensors, face, buttocks

Answer 105

EBV, parvovirus B19 Histo - eccrine gland infiltration with lymphocytes

Answer 106

Virus: Parvovirus B19 single stranded DNA Presentation: Prodrome 7-10 days Slapped cheek Then lacy, reticulated on extremities Or in adolescents/adults - more acral Can get papular purpuric gloves and socks syndrome Enanthem - erosions and petechiae palate, pharynx, tongue Joints Risks: Aplastic crisis Foetal hydrops, anaemia, death - If pregnant woman is exposed in 1st 2 trimesters then serial USS

Answer 107

Virus: HHV6 (sometimes 7) Presentation: Infants, toddlers High fever but otherwise well 3-5 days Exanthem at defervescence Rose-pink macules, papules At trunk, neck, proximal extremities +/- face Enanthem - red papules soft palate + uvula (Nagayama spots)

Answer 108

LL RINHART Congenital leukaemia cutis Congenital Langerhans cell histiocytosis Congenital alveolar rhabdomyosarcoma Infections - coxsackie virus, parvovirus Neonatal neuroblastoma Haemangiomatosis and glomuvenous malformations Haemolytic anaemia - rhesus Rubella and other TORCH (toxoplasmosis, CMV) TORCH stands for: toxoplasmosis, others (syphilis, hepatitis B), rubella, cytomegalovirus, herpes simplex

Answer 109

Virus type: RNA virus Presentation: Incubation 16-18 days Prodrome 1-5 days Rose pink macules 2-3 days Face, cephalocaudal Enanthem - Forccheimer spots = petechial macules on soft palate Joints Complications: Hepatitis Myocarditis, pericarditis Haemolytic anaemia Thrombocytopaenia Encephalitis rare FOETAL INFECTION - worst in 1st 16 weeks - blueberry muffin - cataracts, deafness, congenital heart defects (VSD), CNS (microcephaly, developmental delay) - If pregnant woman exposed - serology, IVIG, prenatal counselling

Answer 110

Virus type: RNA virus Presentation: Incubation 10-14 days Prodrome 2-4 days fever, cough, coryza, conjunctivitis and Koplik spots - grey white papules on buccal mucosa Exanthem 5 days begins at hairline, forehead, behind ears, cephalocaudal Complications: Pneumonia Encephalitis (+ subacute sclerosing panencephalitis) Myocarditis Otitis Treatment: Vitamin A 50,000 - 200,000IU IVIG vaccine Vaccine regime: MMR vaccin age 12 months MMRV at 18 months

Answer 111

Virus: Enterovirus Coxsackie A16 most common Single stranded RNA

Answer 112

Enteroviruses: - Coxsackie A + B - Echoviruses Oral mucosa

Answer 113

Vascular papules Echovirus, less often CMV, EBV

Answer 114

AD MEN1 Multiple facial angiofibromas and collagenomas Lipomas multiple gingival papules confetti-like hypopigmented macules CALM 3 Ps Parathyroid - hyperplasia, adenoma Pituitary - prolactinoma, acromegaly, Cushings Pancreas - adenoma, carcinoma

Answer 115

AD RET Pruritus Lichen amyloidosis, macular amyloidosis, notalgia paraesthetica Thyroid - medullary carcinoma Parathyroid - hyperplasia, adenoma Adrenal - pheochromocytoma

Answer 116

AD RET Multiple mucosal neuromas - Esp conjunctivae, lips, anterior tongue - From birth or early childhood (also in PTEN hamartoma tumour syndrome) Cutaneous neuromas are rare - when present are perinasal Increased nerve fibers in normal skin Everted lips Everted eyelids CALM Hyperpigmentation around mouth and overlying small joints of hands and feet GI - ganglioneuromatosis Thyroid - medullary carcinoma Adrenal - pheochromocytoma Marfanoid

Answer 117

PTEN AD Tumour suppressor gene Trichilemmomas Acral keratoses Mucocutaneous neuromas Oral papillomas Pigmented macules of the glans penis Vascular anomalies, fast flow Lipomas, testicular Increased risk melanoma Other: achrocordons, sclerotic fibromas, inverted follicular keratoses Lhermitte-Duclos, dysplastic gangliocytoma of cerebellum Macrocephaly GI hamartomas Breast cancer Endometrial cancer Thyroid (follicular) cancer Mental/cognitive impairment Colorectal cancer ASD RCC Other: benign ovarian cysts, uterine leiomyomas

Answer 118

APC AD Epidermoid cysts Desmoid tumours Fibrous tumours (skin, mesentery, retroperitoneum) Congenital hyperpigmentation of retinal pigment epithelium (CHRPE) Osteomas Dental anomalies (supernumerary teeth) Increased risk cancers - SHAT PANts Sarcomas Hepatoblastoma Adrenal Thyroid Pancreatic and biliary tract

Answer 119

Peutz-Jeghers Syndrome - mucocutaneous lentigines Juvenile polyposis syndromes - may be associated with HHT PTEN hamartoma tumour syndrome Muir-Torre

Answer 120

AD MSH2 and 6 MLH1 Sebaceous neoplasms (~30%) - Adenoma, sebaceoma or carcinoma Keratoacanthomas Malignancy risk: - adenocarcinoma of colon - followed by genitourinary carcinomas - others: breast, haem, head and neck, small intestine, stomach, pancreas

Answer 121

AR Deficiency of homogentisate 1,2-dioxygenase Skin/eye/cartilage pigmentation - Ears, axilla, sclera Urine darkens on standing Arthritis Valvular heart disease Renal calculi and occasionally failure

Answer 122

AR Alopecia Periorificial erythema and erosions Holocarboxylase - neonates - encephalopathy and hypotonia Biotinidase - young children - developmental delay, hearing loss, seizures

Answer 123

X linked GLA Deficiency of alpha-galactosidase A Angiokeratoma corporis diffusum Pain and paraesthesias of extremities/acral Hypohidrosis Renal and coronary insufficiency Whorled corneal opacities CVA Urine microscopy: birefringent lipid globules (“Maltese crosses”) Enzyme replacement therapy Migalastat Gabapentin (pain) ACEi (proteinuria)

Answer 124

Solitary or multiple Fordyce (scrotum, vulva) Mibelli Circumscriptum Corporis diffusum

Answer 125

AR deficiency of glucocerebrosidase Ichthyosis, colloidion, yellow-brown pigment/easy tanning HSM Cytopaenias Bone disease Variable CNS

Answer 126

AR SLC6A19 (BOAT1) pellagra-like dermatosis associated with photosensitivity ataxia, tremors, nystagmus

Answer 127

AR phenylalanine hydroxylase deficiency Pigmentary dilution of the skin, eyes, and hair (deficiency of tyrosine) Sweat with a musty or “mousy” odor Atopic-like dermatitis Sclerodermatous skin changes Progressive developmental delay early institution of a phenylalanine-restricted diet

Answer 128

LMNA gene de novo dominant mutation Growth failure High pitched voice Premature cardiac disease Macrocephaly Characteristic facies with prominent scalp veins and a beaked nose Wrinkled, thin, dry skin Scleroderma-like Alopecia Perioral cyanosis Irregular brown pigmentation in sun-exposed areas

Answer 129

RECQL2 AR Premature aging Short stature, thin limbs Osteoporosis Cataracts diabetes mellitus and atherosclerosis Skin - atrophy, scale, scleroderma-like, mottled hyperpigmentation keratoses and ulcerations over bony prominences

Answer 130

X linked EDA Hypohidrosis, overheating Alopecia Peg teeth Sebaceous hyperplasia face Eczema-like Normal nails Hoarse voice Saddle nose, full everted lips and frontal bossing May have collodion membrane as baby Immune deficiency variant IKBKG + colitis + arthritis + pyogenic bacterial infections, especially of the skin and respiratory tract

Answer 131

AD GJB6 encodes connexin 30 Normal teeth and sweating Hair wiry, brittle and pale; alopecia Nail dystrophy, small, white Pebbled skin palms and soles

Answer 132

Ichthyoses - CIE, Nethertons, trichothiodystrophy Immunodeficiency - Omens, SCID Infection - staph scalded skin syndrome, congential cutaneous candidiasis Diffuse cutaneous mastocytosis

Answer 133

Intraepidermal blister formation (acantholysis), usually just above basal layer Row of tombstones (loss of desmosomal contact) Early finding - eosinophilic spongiosis DIF - IgG (+/- C3) chicken wire pattern in epidermis

Answer 134

Envoplakin Periplakin DG1, 3 BP230

Answer 135

Acantholysis upper epidermis, within or adjacent to granular layer Eosinophils May also contain neuts in blister cavity DIF - IgG (+/- C3) chicken wire pattern in epidermis

Answer 136

Lichenoid, EM-like, or like PV with suprabasilar acantholysis Keratinocyte necrosis Lymphocytes in epidermis Interface dermatitis Eosinophils rare DIF - IgG and C3

Answer 137

Intraepidermal pustule or vesicle Predominantly neutrophils Usually no acantholysis Subcorneal pustular dermatosis type - subcorneal pustules Intraepidermal neutrophilic type - suprabasilar pustules involving lower or entire epidermis DIF - IgA - upper epidermis in SPD and entire epidermis in IEN

Answer 138

Acral peeling skin syndrome EB simplex Keratolysis exfoliativa Juvenile plantar dermatosis (c/w pitted keratolysis)

Answer 139

Avelumab - PD-L1

Answer 140

Calcium deposition dermis and subcutis vascular (media layer) and extravascular PLUS fibrin thrombi, ischaemic necrosis of overlying epidermis and fat necrosis Stains for calcium: von Kossa, Alizarin red

Answer 141

MDT - renal, pain, wound care, surgery, endocrinology Admit Analgesia, pain team Wound care Discontinue calcium, vitamin D, iron, warfarin, systemic corticosteroids Optimise dialysis IV sodium thiosulfate For high PTH - cinacalcet or parathyroidectomy Phosphate - non calcium based phosphate binders Surgery for limited cautious debridement as needed to promote healing Low threshold to treat infection with abx Apixaban or unfractionated heparin Hyperbaric oxygen Bisphosphonates Pentoxiphyline Vitamin K

Answer 142

2 major, or 1 major and at least 2 minor PASH CORNER Periungual fibromas (2 or more) Angiofibromas (3 or more) Shagreen patch Hypomelanotic macules (3 or more at least 5mm) Cardiac - rhabdomyoma Ocular - retinal hamartomas Renal - angiomyolipoma Neuro - white matter migration lines, SEGAS, astrocytomas, subependymal nodules, cortical tubers (Endocrine - hypothyroid, precocious puberty) Respiratory - pulmonary lymphangioleimyomatosis CORER Confetti skin lesions Oral fibromas Renal cysts Enamel pits Retinal achromic patches

Answer 143

NF SCORE (2 or more) Neurofibromas (2 or more or 1 plexiform) Freckling axillary or inguinal Skeletal - sphenoid wing dysplasia, thinning of long bone cortex CALMs (6 or more, >5mm if pre-puberty; >15mm if post) Optic glioma Relative 1st degree with NF Eyes - Lisch nodules (2 or more)

Answer 144

CALM, neurofibromas, freckling axillary or inguinal JXG Naevus anaemicus Macroglossia and mucosal cobblestoning Pigmentation (hypo, hyper) Connective tissue hypoplasia

Answer 145

BORE(3)D JOGN Breast cancer Optic glioma Rhabdomyosarcoma Endocrine x3 - Phaeochromocytoma - Somatostatinoma - Parathyroid adenoma Duodenal carcinoid JMML Other - CNS - astrocytoma, brain stem glioma GIST or glomus Nerve sheath tumour (MPNST)

Answer 146

Rifampicin 600mg daily for 8 weeks Clarithromycin 500mg BD for 8 weeks

Answer 147

TSC1 - hamartin TSC2 - tuberin

Answer 148

Hypomelanotic macules - first few months Then CALMS Then angiofibromas ~2 years Then shagreen patch ~2 Then periungual fibromas later childhood or adulthood

Answer 149

CALMS Freckling Neurofibromas

Answer 150

TSC NF, Noonan's Fanconi's, Familial Bloom's Leopard, Legius Ataxia Telangiectasia McCune Albright (larger, segmental), Maffuci's Endocrine: MEN2B, MEN1 Also Cowden and Piebaldism

Answer 151

Mx: Full exam including lymph nodes, high res photos, dermoscopy, review in 4 weeks. Remove if progression. Observe if no change. Benign proliferative nodule Neuroid overgrowth/proliferation Melanoma Rhabdomyosarcoma Malignant peripheral nerve sheath tumour

Answer 152

Neurocutaneous melanosis - developmental delay, seizures, headache, vomiting, focal cranial nerve signs, irritability, hydrocephalus (25-35%) Malignant melanoma (10%) Risk Factors: Multiple CMN, satellite naevi Trunk, especailly over spine Larger CMN (esp >40cm) Nodular and rugous Multiple colours MRI imaging if large, multiple naevi and certainly if symtpomatic Other associated symptoms: - Decreased sweating/overheating, skin fragility, erosions, dryness/eczema/itch, fat loss, tenderness

Answer 153

Small <1.5cm diameter (inn adults) Medium 1.5-20cm Large >20-40cm Giant >40cm

Answer 154

Cutaneous mosaicism/ postzygotic genetic mutations NRAS ~80% Other: BRAF, GNAQ, MCIR

Answer 155

MDT - neurology, plastics, counsellor Wound management - dressings, pain relief if ulceration Ix - screen for neurocutaneous melanosis - MRI brain and spine Whole body photography as baseline for surveillance Counsel regarding need for urgent review if new or changing lesions Gen paeds follow up for developmental surveillance 6-12 monthly checks

Answer 156

globular and/or cobblestone pattern coalescing pigmented and non-pigmented papules (monomorphic) hypertrichosis/terminal hairs may get reticular pigmentation may get comma vessels

Answer 157

Infiltrate deep (reticular) Naevus cells/melanocytes surrounding/cuffing blood vessels and adnexal structures and can be found in hair follicles, sebaceous glands, cutaneous nerves, arrector pili muscles etc

Answer 158

Intraparenchymal melanosis (good prognosis) Leptomeningeal involvement/structural abnormalities (poor prognosis)

Answer 159

Early naevus spilus Becker melanosis/naevus Pigmentary mosaicism PIH CMN Masstocytoma

Answer 160

Post zygotic mutation in beta actin Association: smooth muscle hamartoma Becker naevus syndrome - Females > males - Hypoplasia ipsilateral breast, nipple, arm - Enlargement of ipsilateral foot - Skeletal - spina bifida, scoliosis - Supernumerary nipples

Answer 161

AD, mutations in PTPN11 > RAF1 > BRAF Lentigines present in infancy/early childhood Café-noir macules ECG changes (conduction defects, hypertrophic cardiomyopathy), Ocular hypertelorism Pulmonary stenosis Abnormal genitalia growth Retardation Deafness

Answer 162

AD, PRKAR1A Lentigines Atrial Myxoma Blue naevus Risks: testicular cancer (Sertoli), thyroid, pancreas/pituitary

Answer 163

Peutz Jeghers AD, SKT11 - Lentigines perioral, oral mucosa, hands - Longitudinal melanonychia - Hamartomatous GI polyps - Pancreatic carcinoma, ovarian and testicular tumours, breast, GI Laugier Hunziker Sporadic Similar to above without cancers + genital melanosis

Answer 164

Dermal pigment appears blue Decreased reflectance of light in longer wavelength

Answer 165

Spindle shaped pigmented elongated dendritic melanocytes in dermis

Answer 166

Ophthalm - glaucoma, ocular melanoma risk Surveillance low risk melanoma Cosmesis - QS ndYAG or pico (other: ruby, alexandrite)

Answer 167

GNA11, GNAQ Elongated spindle shaped dendritic melanocytes in dermis Often melanophages Sclerosis of collagen Cellular type: more plump melanocytes Uniform slate blue colour, without vascular features or pigment network Sclerotic blue naevus - central pale grey hyperpigmentation (peripherally slate blue)

Answer 168

Globular - child, young adult Reticular - adult Homogeneous - older adult Evolving - symmetrical rim of uniform globules, centrally uniform reticular or homogeneous (be suspicious if >50 or centrally non-uniform)

Answer 169

Parallel furrow Lattice (insteps) Fibrillar (pressure points) - multiple thin parallel lines of pigmentation perpendicular to dermatoglyphs

Answer 170

Cobblestone pattern of pigmented globules which are variably sized but uniformly pigmented, angulated and smooth cornered May be hyperpigmented and have keratin-filled crypts Comma vessels Central perifollicular hyperpigmentation in darker skin

Answer 171

Symmetrical, sharply demarcated Wedge shape (upside down pyramid) Epidermal hyperplasia Rete ridges clutching melanocytic nests Melanocytic nests (junctional, dermal, compound) spindle shaped or epithelioid Clefts around these nests Can be pagetoid Kamino bodies - basement membrane material (eosinophilic, collagen) Maturation of melanocytes on descent (smaller and less atypia) Lymphocytes HMB45 more positive in superficial portion (rather than in melanoma it is superficial and deep)

Answer 172

Pigmented: Pigment globules, surrounded by inverse/negative pigment network, dotted vessels Hypomelanotic: Dotted, coiled, glomerular vessles Negative network OR bland, milky, pinkish erythema and otherwise featureless Reed naevus = heavily pigmented version of spitz naevus Globular, then reticular, then homogenous (with circumferential pigment streaks / starburst)

Answer 173

Treponema pallidum Non-venereal treponematoses: Pinta, Bejel, Yaws Other spirochetes: Borellia, Leptospirosis

Answer 174

10-90 days (avge 3 weeks) Indolent papule, indurated ulcer (chancre), enlarged regional nodes Heals in a few weeks Ulceration Diffuse dermal infiltrate plasma cells, lymphocytes, histiocytes Endothelial swelling Spirochetes - Warthin-Starry or IHC

Answer 175

3-10 weeks after primary Malaise, fever, generalised LAD, sore throat, weight loss, headache Roseola-like macules, non-pruritic papulosquamous eruption, copper coloured Palms and soles - colarette of Biett Mucosal ulcers Condyloma lata Annular/figurate plaques face with central hyperpigmentation Moth eaten alopecia Split papules oral commissures Necklace of venus Corona veneris Crusted necrotic lesions Granulomatous nodules, plaques Lues maligna variant (like disseminated primary chancres) Lasts 3-12 months ~25% relapse ~1/3rd go on to develop tertiary syphilis Great variability on histo § Epidermis may be normal, psoriasiform, necrotic or ulcerated Dermal infiltrates of plasma cells, lymphocytes and histiocytes can be perivascular, lichenoid, nodular, or diffuse Can be granulomatous changes Endothelial swelling and vascular proliferation Lues maligna is characterized by vasculitis Spirochetes - IHC

Answer 176

1/3rd patients Months to years CNS and cardiovascular Also skin, bones Gumma - noduloulcerative, arciform pattern, involute with scarring slowly Histo Tuberculoid granulomas (with or without caseation) are present together with plasma cells Endothelial swelling Organisms may be difficult to identify

Answer 177

Diplopia Lightning pains Loss of vibratory and position sense Reduced reflexes legs Ataxia Sphincter dysfunction Argyll Robertson pupils Visceral crisis

Answer 178

Asymptomatic, meningeal, parenchymatous (including tabes dorsalis), gummatous

Answer 179

Cachexia Skin lesions like acquired secondary Snuffles (bloody purulent mucinoous nasal discharge) Perioral, perianal fissures (rhagades) LAD, HSM Skeletal (osteochondritis) Haem (anaemia, thrmbocytopaenisa) Pneumonitis, hepatitis, nephropatht, neurosyphilis

Answer 180

Child or adolescent Hutchinson triad = Keratitis + dental (Hutchinson teeth) + deafness (neural) (eyes, teeth, ears)

Answer 181

Serology: Cardiolipin (non-treponemal, non-specific) VDRL, RPR Quantitative evaluation useful - fourfold decrease in Ab titre suggests successful treatment; fourfold increase suggests relapse or reinfection Treponemal antigens (specific) TPHA, TPPA Confirms non-treponemal tests Quantitative evaluation NOT useful Dark field microscopy - motile spirochetes PCR Histo

Answer 182

Early Benzathine benzylpenicillin 2.4 million units IM ssingle dose Late latent Benzathine benzylpenicillin 2.4 million units IM weekly for 3 weeks Tertiary Benzylpenicillin 1.8g IV 4hrly for 15 days Congenital Benzylpenicillin 30mg/kg IV for 10 days, BD if <7 days old, otherwise TDS Note: can get Jarisch Herxheimer esp if CVS or neurosyphilis - can give concurrent pred to minimise

Answer 183

Neisseria gonorrhoeeae Gram negative (pink) diplococci

Answer 184

Azithromycin 1g PO PLUS Ceftriaxone 500mg IM or IV Single dose

Answer 185

Haemophilus ducreyi Gram negative (pink) coccobacilli - school of fish or railroad track pattern Papule to pustule to ulcer, can get multiple, undermined edge, sharply demarcated Painful inguinal buboes may rupture and cause inguinal ulceration Azithromycin 1g PO or ceftriaxone 500mg IM or IV

Answer 186

Chlamydia trachomatis Papule/vesicle/ulcer mucosa Inguinal lymphadenopathy Firm mass and bubo - spontaneous drainage and involution Proctocolitis, perirectal abscess, anal fistulas Doxy 100mg BD for 3 weeks

Answer 187

Klebsiella granulomatis Gram negativee bacillus Long incubation up to 1 year Starts papule, ulcers, not very painful, beefy red, tendency to bleed, tissue destruction Histo: Ulceration Pseudoepitheliomatous hyperplasia Histiocytes, plasma cells, few lymphocytes, neutrophils Organisms within histiocytes Donovan bodies - safety pin (bipolar staining of organism) Stains - Giemsa, Wright Treatment: Doxy (100mg BD for 4 weeks) or azithro (500mg daily for 7 days)

Answer 188

Sclerodema, scleromyxoedema Carcinoid Lipodermatosclerosis Eosinophilic fasciitis Radiotherapy induced Occupational/drugs (epoxy resins, vinyl chloride, silica) Genetic - stiff skin and ageing syndromes Autoimmune/sclerodermoid GVHD Metabolic (PCT, nephrogenic systemic fibrosis, PKU) Endo (POEMS, diabetic cheiroarthropathy, myxoedema) Systemic amyloidosis

Answer 189

Fingers - thickening of fingers proximal to MCPs - 9 points - puffy fingers - sclerodactly - raynauds Capillary nail fold abnormalities Antibodies - specific to SSc (centromere, Scl-70/anti-topoisomerase 1, RNA polymerase 3) Pulmonary - ILD or PAH Ulcers digits tips (or finger tip pitting scars) Telangiectasias

Answer 190

Limited: Anti centromere Raynauds many years PAH Sclerosis distal (+ face) only Slow progression Diffuse: Anti topoisomerase 1 and RNA polymerase 3 Sudden onset Raynauds ILD Renal (HTN, renal crisis) Sclerosis distal but also proximal >90% demonstrate internal organ involvement within 5 years Common to both: Dysphagia Telangiectasias Nail fold capillary changes Cardiomyopathy/heart failure

Answer 191

Pruritus Beaked nose Microstomia Sicca symptoms Subsequent dental issues Decreased facial expression amimia Dyspigmentation - "Salt and pepper" sign or leukoderma of scleroderma - Localised areas of depigmentation with sparing of perifollicular skin Calcinosis cutis

Answer 192

Compact, hyalinised collagen; excessive deposition of collagen Atrophic eccrine and pilosebaceous glands Loss of subcutaneous fat Sparse lymphocytic infiltrate in dermis and subcutis Adnexal structures, especially eccrine glands, appear 'trapped' by the excessive deposition of collagen

Answer 193

General - Avoid trauma - Keep warm - Physio, OT, stretch and exercise, massage - Heat, wax baths - Skin care - Scleroderma Australia Topicals - potent TCS PUVA/UVA1, nbUVB Systemics - MMF - 1st line - Cyclophosphamide - HCQ (not in CME) - Caution with pred (scleroderma renal crisis) - MTX (skin benefits only) HSCT with careful patient selection, significant treatment-related toxicities Emerging/new - Nintedanib, tyrosine kinase inhibitor - JAKi - tofa - IL6 - Transforming growth factor beta inhibition - B cell depletion - rituximab - Abatacept Treatments for Raynauds and calcinosis cutis and digital ulcers - Minimise cold, prevent trauma, quit smoking - Double lined gloves, heat pads - Analgesia - Meticulous wound care - Calcium channel blockers - PDE5 (sildenafil) - also helps PAH - Prostanoids, iloprost - SSRIs, ARB2, ACEi, anticoagulants, statins, alpha blockers - Bosentan - Botulinum toxin injection - Sympathectomy Telangiectasias - Camouflage, PDL, IPL, fine wire diathermy Treatments for itch - Antihistamines - Montelukast - Menthol - Gabapentin, pregabalin, doxepin, naltrexone - Cannabinoid receptor modulator ACEi - cardiac and renal Nutrition supplementation for malabsorption GORD Rx

Answer 194

Nucleolar or speckled

Answer 195

Rodnan Skin Score Patient reported outcomes HAQ-DI - Health Assessment Questionnaire-Disability Index Combined response index for systemic sclerosis (CRISS)

Answer 196

dcSSc 5 year survival = 85% lcSSc = 95% Poor prognostic factors: Male Older age Diffuse Organs (ILD, PAH, SRC, cardiac) Digital ulcers Joint involvement

Answer 197

Morphoea has no: Nailfold capillary abnormalities Sclerodactyly Fibrosis of internal organs - can get arthralgia and GORD but this is due to systemic inflammation not fibrosis SSc specific antibodies Raynauds uncommon

Answer 198

RNAP3 Lung, haem, liver, bladder, breast

Answer 199

ILD, PAH, CCF, valvular disease, pericardial effusions, arrhythmias - PFTs, echo, ECG, HRCT, troponin, BNP SRC - BP, UECs, urinalysis GI - impaired oesophageal mobility, GORD - Barrium swallow, endoscopy Malignancy Sexual dysfunction

Answer 200

RAMBED Retinoids Anorexia Menkes - XLR ATP7A Bjornstad syndrome, Bazex Dupre Ectodermal

Answer 201

RAYNAUDS Repetitive trauma, vibration, cold injury Arterial - Buerger's, atherosclerosis Y - idiopathic (or primary) Neurologic, neoplastic - Carpal tunnel, thoracic outlet syndrome, carcinoid, phaeochromocytoma APLS Underlying haem abnormality - cryoglobulinaemia, cold agglutinin, myeloproliferative (essential thrombocythaemia) Drugs - BECOM (cold) Beta blockers, bleomycin and other chemo, ergot alkaloids, clonidine, CsA, cocaine, OCP, metals SSc/SLE/DM/SjS/mixed

Answer 202

SOB CVC Smoking OCP Beta blockers Caffeine Vibration Cold

Answer 203

Hx strenuous activity Hypergammaglobulinaemia pancytopaenia, anaemia, thrombocytopaenia, myeloproliferative disorders, monoclonal gammopathy Insect bites, radiation, infections, medications

Answer 204

Pred 1-2mg/kg - Tapered over 6-24 months HCQ Cyclosporine Dapsone MTX PUVA Infliximab Physio

Answer 205

Red and white structureless areas White lines Difficult to distinguish from BCC, easy to distinguish from SCC Absence of white circles Absence of opaque yellowish-white scales / pigmented structures Absence of arborising/hairpin vessels

Answer 206

Dermal proliferation bizarre spindle cells, epithelioid cells, multinucleated giant cells, extending up against epidermis Severe pleomorphism, hyperchromatism, many atypical mitoses Solar elastosis Stains: CD10 and vimentin positive (also procollagen 1, CD68 and other histiocytic stains) Negative for (ddx): SOX10, S100, p40, desmin, CD34

Answer 207

Folliculitis Acneiform eruptions Alopecia Hypertrichosis Trichomegaly Xerosis Paronychia Skin cancer

Answer 208

- Interactions - CYP450 3A4 inhibitor - Pregnancy category A (?cardiovascular malformations ?pyloric stenosis) - Nausea, abdo pain, diarrhoea - Reversible hearing loss high doses - QT prolongation - Hepatitis Comes in 250mg tablets 250mg BD-QID or 500mg BD

Answer 209

Programmed cell death protein 1 - PD1 Nivolumab Pembrolizumab Cytotoxic T lymphocyte associated antigen 4 - CTLA4 Ipilimumab Programmed death ligand 1 - PDL1 Avelumab (used for Merkel cell carcinoma) LAG3 - relatlimab - new Rx for melanoma Pruritis Maculopapular Lichenoid drug eruption Vitiligo Psoriasis Bullous pemphigoid SCAR Sarcoid Neutrophilic - Sweets

Answer 210

Mitogen-activated protein kinase extracellular receptor kinase (MEK) inhibitors Trametinib Imatinib Sorafenib Dasatinib Binimetinib Cobimetinib Acneiform eruption Pruritus Xerosis Alopecia Peripheral oedema Pyrexia, fatigue, GI upset, arthralgias, abnormal liver enzymes, cardiovascular and renal toxicity, neuro disorders

Answer 211

prevent overactivation of cellular proliferation by inhibiting kinase activity Dabrafenib Vemurafenib Hyperproliferative epidermal neoplasms in up to 99% of patients on BRAF inhibitor monotherapy within first 6 months - AKs, KAs, verrucal or plantar hyperkeratosis, SCCs Eruptive melanocytic naevi UVA mediated photosensitivity KP-like eruptions Grover's Palmoplantar erythrodysaesthesia (hand and foot syndrome) Morbilliform drug eruption Pyrexia, fatigue, GI upset, arthralgias, abnormal liver enzymes, cardiovascular and renal toxicity, neuro disorders

Answer 212

Annular erythema and blisters - string of pearls/crown of jewels Flexures, lower trunk, thigh, groin Herpetiform/grouped Tense bullae Mucous membranes in ~70% - esp ocular, also oral (tongue, gingiva), nasal, pharyngeal, oesophageal TEN-like 1/3 drug induced VPCANS Vancomycin Penicillins, phenytoin Captopril, cephalosporins ACEi, ARBS, amiodarone NSAIDs Sulfonamides (also lithium, atorvatstain, frusemide) 2/3 spontaneous or other trigger incl infection - URTI, tetanus, VZV Other associations: IBD Malignancy - B cell lymphoma, CLL, solid (bladder, thyroid, colon, oesophagus) AICTD - SLE, RA, DM, thyroid

Answer 213

Subepidermal split Neutrophilic infiltrate papillary dermis Linear IgA along DEJ

Answer 214

TCS Dapsone (quick response 48-72hrs) Sulfapyridine Colchicine Tetracyclines Erythromycin MMF, MTX, AZA, CsA Rituximab IVIG

Answer 215

HLADQ2 (90%), HLADQ8 (~10%) Gliadin peptide (DGP) IgG Tissue transglutaminase (tTG) IgA IgA FBE, UEC, LFTs, CMP, Fe studies, B12, folate, zinc, TFTs (incl antibodies) Scopes with gastro 15-20% of pts with coeliac disease get DH >90% of pts with DH have gluten sensitive enteropathy (only ~20% have symptoms)

Answer 216

Pruritic papulovesicles, grouped Erythematous base Excoriation, crusting Can be pleomorphic - urticarial, papules, vesicles Extensor surfaces - elbows, knees, scalp, buttocks Prurigo PIH Haemorrhagic acral macules Digital petechiae Also examine for signs of anaemia

Answer 217

Autoimmune thyroid IDDM/T1DM AA Vitiligo Pernicious anaemia Addisons Haemochromatosis Recurrent miscarriage Fatty liver Pericarditis/cardiomyopthy Dental Neuro (polyneuropathy, epilepsy) Non Hodgkin Lymphoma intestines (6-10x risk) (gluten fee diet is protective from enteropathy associated T cell lymphoma)

Answer 218

Subepidermal bullae with neutrophilic infiltrate Multilocular neutrophilic abscesses in dermal papillae Superficial perivascular lymphocytic infiltrate DIF - granular IgA dermal papillae (perilesional skin)

Answer 219

Dapsone - lack of GI response - skin improvement within 48-72 hrs - recurrence within 24-48 hrs Gluten free diet - reduced need for meds - improves enteropathy - reduces risk of lymphoma - improves nutrition and bone density Gluten is in wheat, rye, barley, sometimes oats Other: Steroids Colchicine CsA AZA Sulfpyridine Rituximab

Answer 220

PA NO K SON(S) Papules - "greasy, keratotic, crusted" - seborrhoeic, intertriginous, periauricular, palmoplantar - acrokeratosis veruciformis of Hopf like Pits - keratin-filled Acral haemorrhage Nails - longitudinal erythronychia - longitudinal ridging - wedge-shaped subungual hyperkeratosis - v shaped notches - brittle nails Oral mucosa - papules, hard palate Kaposi varicelliform + infections - HPV, HSV, bacteria, yeast, dermatophytes - malodour Salivary gland obstruction - painful glandular swelling Ocular - rare - corneal ulceration, staph endophthalmitis Neuropsychiatric - epilepsy, mood disorders, intellectual impairment SCC - chronically affected areas

Answer 221

Avoid triggers - light weight clothing, sunprotection Antimicrobial cleansers Clindamycin 1% lotion daily TCS - advantan cream daily Topical CNI Topical retinoids Keratolytic emollient Systemics - isotretinoin, acitretin Surgical excision CO2 laser, erbium YAG Dermabrasion PDT, PDL

Answer 222

Benign familial pemphigus Moist, malodorous vegetations, fissures, rents or flaccid blisters, vesicles, erosions Axillae and groin Lateral aspects neck and perianal Scalp, antecubital and popliteal fossa, trunk Nails - longitudinal leukonychia Pain, itch, odour Cx: kaposi varicelliform eruption, SCC

Answer 223

Lightweight clothing AAntimicrobial cleansers nd topicals eg clindamycin 1% lotion Advantan cream daily ILCS Top CNI Chemodenervation with botulinum toxin Zinc oxide paste Dermabrasion CO2 laser, erbium YAG PDT Surgical excision and grafting Doxycycline Variable efficacy with retinoids Naltrexone (case reports dapsone, CsA, MTX)

Answer 224

exfoliative toxins A and B - bind and cleave desmoglein 1 granular layer

Answer 225

small neutrophilic vesiculopustules in epidermis - gram +ve cocci within spongiosis intense infiltrate of neutrophils and lymphocytes in dermis typically cleavage within the granular layer

Answer 226

clindamycin 300-450mg QID (also option for MRSA) - or bactrim

Answer 227

12.5mg/kg QID

Answer 228

Occlusion, maceration, and hyperhydration of the skin Shaving, plucking, or waxing hair Use of topical corticosteroids Hot and humid weather Atopic dermatitis Diabetes mellitus

Answer 229

Abscess can occur anywhere Furuncle involves a hair follicle Carbuncle = a contiguous collection of furuncles

Answer 230

close personal contact with affected individuals (e.g. households, athletic activities) diabetes mellitus obesity poor hygiene immunodeficiency syndromes (e.g. chronic granulomatous disease, hyperimmunoglobulin E syndrome) Furuncle - MRSA

Answer 231

Exotoxin called toxic shock syndrome toxin-1 (TSST-1)

Answer 232

Tampons Surgery (packing, meshes) Nasal packing insulin pump infusion sites Burns Abscesses postpartum infections Contraceptive sponges

Answer 233

Diffuse erythema or scarlatiniform Trunk to extremities Erythema and oedema palms and soles Strawberry tongue, erythema oral mucosa Conjunctival erythema Generalised non pitting oedema Desquamation hands and feet onychomadesis, Beau's lines, telogen effluvium Renal impairment Weakness, fatigue, myalgias, arthralgias Strep: Severe local pain in extremities More blistering Less erythema ARDS Much higher mortality 30-60%

Answer 234

Streptococcal pyrogenic exotoxins (SPEs) ABC For strep toxic shock also streptococcal mitogenic exotoxin Z (SMEZ)

Answer 235

ICU IV fluid, vasopressors Foreign bodies/source removed if staph, early surgical intervention if strep IVIG Penicillin/fluclox + clindamycin Vanc if MRSA staph decolonisation future

Answer 236

Group A strep, other strep, S. aureus, Pneumococcus spp., Klebsiella pneumoniae, Yersinia enterocolitica, and Haemophilus influenzae type Treatment Penicillin V 500mg QID for 5 days Benzylpenicillin IV if severe

Answer 237

7-day course of cefuroxime > 10-day course of penicillin

Answer 238

1-10yo usually follows tonsillitis or pharyngitis sudden onset of a sore throat, headache, malaise, chills, anorexia, nausea, and high fevers eruption begins 12–48 hours later erythema neck, chest, and axillae then generalised tiny superimposed papules with a sandpaper-like texture (“sunburn with goose pimples”) Pastia's lines (linear petechial streaks) - axillary, antecubital, and inguinal areas flushed cheeks with circumoral pallor desquamation esp hands and feet beefy red strawberry tongue throat red, oedematous, exudate palatal petechiae tender cervical adenopathy

Answer 239

penicillin V 15mg/kg BD (or amoxicillin) - 10-14 days (prevents development of rheumatic fever) If penicillin allergy - first-generation cephalosporin, clindamycin analgesia otitis, mastoiditis, sinusitis, pneumonia, myocarditis, meningitis, arthritis, hepatitis, acute glomerulonephritis, and rheumatic fever

Answer 240

petechiae and splinter hemorrhages Osler nodes - tender, erythematous papules and nodules with white centers - finger pads and thenar/hypothenar eminences Janeway lesions - painless, small hemorrhagic macules or papules on the palms and soles

Answer 241

Gram positive rod Erythrasma Pitted keratolysis (more commonly Kytococcus sedentarius though) Trichomycosis Axillaris, Pubis, and Capitis

Answer 242

Optimise risk factors 20% aluminum chloride TOP clindamycin, erythromycin, mupirocin, fusidic acid, and Whitfield ointment (salicylic 3% and benzoic 6% acids) Oral erythromycin, tetracyclines, or single-dose clarithromycin 1g stat can be used for widespread or recalcitrant cases Antimicrobial wash

Answer 243

Erysipelothrix rhusiopathiae Gram positive bacilli fishermen or persons who prepare meat, poultry, or fish Penicillin is the drug of choice; erythromycin, cephalosporins, clindamycin, linezolid, and quinolones are alternatives Wear gloves

Answer 244

Neisseria Meningitidis aerobic Gram-negative diplococcus produces endotoxin shock, multi-organ failure, and purpura fulminans Fever petechial eruption Chills Myalgias Headache Then retiform purpura and ischemic necrosis Bullous hemorrhagic lesions Hypotension, meningitis, meningoencephalitis, pneumonia, arthritis, pericarditis, myocarditis, disseminated intravascular coagulation (DIC)

Answer 245

third-generation cephalosporin (e.g. cefotaxime, ceftriaxone) - while awaiting sensitivities All close contacts should receive prophylactic treatment with ciprofloxacin, rifampin, or ceftriaxone Blood and CSF culture Notifiable Droplet precautions 24hrs Vaccine: ACWY vaccine - 12 months old - 14-16 years (can catch up to age of 20) - asplenia/hyposplenia, complement deficiency and those receiving treatment with eculizumab B vaccine - Aboriginal and Torres Strait Islander children aged 2 months, 4 months, 6 months (eligible medical conditions) and 12 months (can catch up to age of 2) - asplenia/hyposplenia, complement deficiency and those receiving treatment with eculizumab

Answer 246

Chloronychia Folliculitis (hot tub) Ecthyma Gangrenosum Pseudomonal Pyoderma Otitis Externa and Malignant Otitis Externa Pseudomonas Hot-Foot Syndrome

Answer 247

Bartonella bacilliformis Bartonellosis - Oroya fever and verruga peruana (Peruvian wart) Bartonella henselae Cat Scratch Disease Bacillary Angiomatosis Bartonella quintana Bacillary Angiomatosis

Answer 248

Vinegar soaks (1:5) 2% sodium hypochlorite (household bleach diluted 1:4) Quinolone (ciprofloxacin) Or if on more serious end of spectrum: intravenous antipseudomonal penicillins or cephalosporins as well as oral ciprofloxacin If pseudomonal folliculitis: resolves spontaneously in 7–14 days Warm compresses with 2% acetic acid Topical polymyxin B or gentamicin Oral quinolone if severe

Answer 249

Neutrophilic eccrine hidradenitis Idiopathic palmoplantar hidradenitis Pseudomonas hot foot Erythema nodosum Medium vessel vasculitis Pernio

Answer 250

Burkholderia pseudomallei Gram negative bacilli Environmental sprophytes found in soil Cutaneous findings in 10-20% of melioidosis Primary or secondary 1 - solitary non-healing ulcer, often leg However large spectrum of presentations (abscesses, cellulitis, pustules, boils, carbuncles, echthyma like, nec fasc) Risk factors (more for systemic): DM Male ETOH CKD Immunosuppression Malignancy Chronic pulmonary disease Organs: Lungs, spleen, liver, prostate, kidneys, bones, joints, muscles, brain

Answer 251

Investigations: Bacterial MCS - bipolar stain, safety pin PCR Histo - necrotising to granulomatous inflammation Blood and urine cultures CXR +/- CT CAP, MRIB Hospital admission, MDT IV Non-neurological - ceftazidime or meropenem Neurological - meropenem 10-14 days Then an eradication phase >3 months bactrim + folic acid

Answer 252

Malaise, night sweats Bacillary peliosis hepatitis and splenis Histo: lobular proliferation capillaries and venules neutrophils clumps of bacilli in interstitium positive warthin starry B Haensle PCR Mx: At least 3 months abx Mild - doxy or erythro Severe - doxy + gentamicin +/- surgery

Answer 253

Clinical: Involvement of webspace, sparing of terminal phalanges Complications: Endocarditis Septic arthritis Visceral/cerebral abscess Classification: - localised - diffuse cutaneous - systemic (arthralgia, fever) Diagnosis: - Culture difficult - PCR swab/tissue

Answer 254

Coral red Coproporphyrin III

Answer 255

2 major, or 1 major and 2 minor (accompanied by evidence of an antecedent group A streptococcal infection) Major - SPACE Subcutaneous nodules Pancarditis Arthritis Chorea (Sydenham's) Erythema margintum Minor - PEACE Previous hx of rheumatic fever or RHD ECG with prolonged PR Arthralgias CRP/ESR elevation Elevated temperature

Answer 256

Seen in <10% of patients with RF 2-5 weeks post strep throat Erythematous macule to patches, plaques Polycyclic/serpiginous borders Asymptomatic Migratory (2-12mm over 12 hours) Last hours to days Most noticeable in the afternoon Resolves with hypopigmentation Trunk, axillae, proximal extremities, spares face

Answer 257

AD GJB3 or 4 Fixed and migratory plaques (geographic) PPKD Will have been present from birth or soon after (50%) or within first year of life (90%) On poikiloderma list, annular list, and 'ichthyosis' list (scaly, patterned, red, isolated) - but really an erythrokeratoderma

Answer 258

superficial lesions - mild spongiosis and microvesiculation with associated focal parakeratosis perivascular lymphohistiocytic infiltrate, tight aggregate of inflammatory cells around vessels (coat sleeve) can be superficial or deep

Answer 259

Pregnancy Foods - blue cheese, tomato Fungi - candida, tinea A - worms (ascaris) Sjogrens Thyroid Malignancy (lymphoma, leukaemia) Infection - HIV, hepatitis IBD Liver disease Drugs - NSAIDs, diuretics,

Answer 260

Firm pink papules that expand centrifugally and then develop central clearing Last days to months If superficial - classic trailing white scale Deep have more infiltrated border Thighs, hips can enlarge to greater than 6 cm in diameter over a period of 1 to 2 weeks Rx: Symptomatic TCS, topical CNI nbUVB Antihistamine Rare to need systemic agent, some might consider antibiotic

Answer 261

migratory composed of concentric rings wood-grain appearance rapid migration (up to 1 cm per day) may also get PPKD and ichthyosis

Answer 262

paraneoplastic phenomenon (in >70-80%) Lung breast, oesophagus, stomach TB

Answer 263

hyperkeratosis, focal parakeratosis, moderate patchy spongiosis and a mild, perivascular lymphohistiocytic infiltrate

Answer 264

Borrelia burgdorferi Ixodes tick Hosts: mice, chipmunks, birds, deer Erythema migrans = initial cutaneous manifestation of Lyme disease - seen in 60-90% (Early localised phase of Lyme disease, as opposed to early disseminated and then chronic) 7-14 days post tick detachment Erythematous, expanding, circular or annular plaque lighter-coloured central area or bull's-eye appearance centre - darker red to violaceous in colour, crusted, or even vesicular Eventual diameter is usually at least 5 cm Lasts 4 weeks untreated

Answer 265

superficial and deep lymphoid infiltrates, a few eosinophils and plasma cells Apoptotic cells in epidermis reduction in number of Langerhans cells PCR analysis of tissue specimen - Borrelia spirochetes Stain: Warthin-Starry Serology - IgM response occurs 3-6 weeks into infection ELISA, Western blot

Answer 266

Arthritis (often knee) Neuropathy (most commonly facial nerve palsy) Encephalopathy Cardiac (pancarditis, AV block)

Answer 267

Doxy 100mg BD for 14 days

Answer 268

Scarlet fever Rheumatic fever Post strep GN Guttate psoriasis Strep toxic shock syndrome Bacteraemia OM Septic arthritis IE

Answer 269

Gram positive branching filaments Acid-fast positive Mycetoma (sulfur granules) Lymphocutaneous/sporotrichoid Superficial cutaneous Systemic/disseminated (immunosuppressed) sulfonamides, mino

Answer 270

Gram positive branching filaments Acid-fast negative poor dental hygiene, dental disease, or a previous orofacial injury or procedure Cervicofacial actinomycosis - suppurating abscesses, granulomatous inflammation, and sinus formation Sulfur granules Pulmonary/thoracic GI Pelvic penicillin G or ampicillin

Answer 271

Congo red Crystal violet Methyl violet PAS (periodic acid Schiff) Sirius red Pagoda red Dylan stain Thioflavin T

Answer 272

Primary - AL (amyloid light chains - containing immunoglobulin light chains) Secondary - AA (amyloid-associated - composed of an acute phase protein synthesised by the liver)

Answer 273

DAMPS Dyskeratosis congenita Autoimmune - SLE, DM, PBC, SSc MEN2A Paraproteinamia Sjogrens

Answer 274

Epidermis - acanthosis, compact orthohyperkeratosis (from rubbing) Macular and lichen - amyloid deposits restricted to upper dermis Lichen - deposits may expand the papillae and displace the elongated rete ridges melanophages and sparse perivascular lymphohistiocytic infiltrate fissures Nodular - diffusely infiltrated with deposits - dermis, subcutis, blood vessel walls

Answer 275

Breaking the itch-scratch-itch cycle (avoid triggers: heat, sun) Potent topical steroid - Under occlusion in lichen amyloidosis Addition of SA if lichen amyloidosis Avoid scratching/rubbing with occlusive dressings - hydrocolloid or gauze wraps impregnated with zinc nbUVB or PUVA Dermabrasion CO2 or erbium:YAG laser Acitretin (0.5mg/kg/day) ?cyclophosphamide Nodular - surgical excision, cryotherapy, electrodessication, CO2 laser - may recur Physiotherapy Dimethyl sulfoxide (DMSO)

Answer 276

Underlying plasma cell dyscrasia fatigue, weight loss, paraesthesias, dyspnoea, syncopal attacks Macroglossia + carpal tunnel Skin (involved in ~25%): Waxy, translucent or purpuric papules, nodules and plaques Smooth skin coloured papules at face (often around eyelids), neck, scalp, anogenital region Leonine facies Oral - macroglossia with scalloping, xerostomia, soft rubbery swellings, haemorrhagic papules, plaques and blisters Petechiae, purpura, ecchymoses - especially eyelids (racoon eyes), neck, axillae, anogenital region Sclerodermoid scalp folds resembling cutis verticis gyrata, with alopecia Nail dystrophy Haemorrhagic bullous lesions Other: Renal - nephrotic syndrome - Proteinuria, hypoalbuminaemia, oedema Cardiac - restrictive cardiomyopathy or CHF Autonomic and sensory neuropathies Hepatomegaly

Answer 277

FBE, UEC, LFTs Myeloma screen - SPEP, UPEP, free light chains Bone marrow biopsy Random biopsy if no skin lesions: - random rectal mucosal biopsy, abdominal fat FNA Otherwise biopsy skin lesions Screen for systemic involvement: Urine ECG, echo, troponin, BNP NCS if sx

Answer 278

E.g. Muckle Wells, familial cold urticaria/inflammatory syndrome NLRP3 Muckle Wells Familial urticaria in early childhood Fever, limb pains, urticaria, progressive perceptive deafness AA nephropathy 25% a/w amyloidosis Cold triggered

Answer 279

most common monogenic autoinflammatory syndrome AR MEFV Recurrent febrile episodes, pleuritis, peritonitis, synovitis Large joint arthritis Acute scrotal swelling Erysipelas like erythema - joints, lower legs, dorsal feet Small vessel vasculitis Non-specific purpuric lesions Risk developing AA amyloidosis - largely prevented with continuous use of colchicine Colchicine

Answer 280

KINKY PLIGHT Menke's Kinky Hair Disease Pityriasis lichenoides chronica Lichen sclerosis Linear naevoid hypopigmentation Lichen striatus Incontinentia pigmenti Goltz Conradi Hunermann Happle Tuberous sclerosis Other: segmental vitiligo, segmental pigmentation disorder, epidermal naevus, pigmentary demarcation lines,

Answer 281

Segmental: uni- bi- or pluri- segmental Non segmental: Acrofacial Generalised Universal (>80% BSA) Mucosal Mixed (NSV and SV) Rare variants - follicular; vitligo minor (hypochromic); vitiligo punctata; inflammatory Undetermined or unclassified: Focal - small isolated patch does not fit into segmental pattern Mucosal site in isolation

Answer 282

Acquired chronic depigmentation disorder Loss of functional melanocytes Affects 0.5-1% of the population Autoimmune

Answer 283

3 most common: Thyroid (15-20%) Addisons T1DM Others: Pernicious anaemia Coeliac disease Cochlear dysfunction - hearing loss Atopic dermatitis SLE, RA Uveitis Routinely screen for TFTs and antibodies Anti thyroid peroxidase Antithyroglobulin Not recommended to test further: ANA, FBE, B12, UECs, BSL

Answer 284

Inherited/genetic: Piebaldism TS Hypomelanosis of ito Waardenberg Griscelli Menkes PIH: Eczema Psoriasis Lichen planus Pit alba LS Paramalignant: MF Melanoma associated Parainfectious: Leprosy Tinea versicolour Syphilis and other treponemal infections Onchocerciasis Drug induced: Potent TCS Imiquimod Imatinib Naevus depigmentosis Idiopathic Guttate Hypomelanosis Trauma/burn/scar

Answer 285

Establish: Type, site, extent of vitiligo Skin PT Age of onset, stability, speed of onset Type and duration of prev Rx Triggers QoL and psychosocial impact Hx of autoimmune/thyroid disease Routinely screen for TFTs and antibodies Photos Scores - Patient Health Questionnaire 4 or 9, Generalised Anxiety Disorder 7 (GAD7), DLQI, Vitiligo Impact Patient Scale (VIPS) Education Discussion around psychosocial impact Sunprotection Vitamin D Cosmetic camouflage Potent or v potent TCS (alternating regimens option) Tacro for face nbUVB (counsel) PO dexa 4mg on weekends if rapidly progressive Excimer laser if localised Cellular grafting if stable (offer) Depigmentation therapies when extensive (offer) CO2 laser + 5FU for nonsegmental vitiligo on hands - once daily for 7 days per month for 5 months Review 3-4 monthly with photos and scores

Answer 286

Trichrome Koebnerisation Leukotrichia (white hair)

Answer 287

melanocytes are typically absent or present in very small numbers IHC: Melan-A, HMB45

Answer 288

KIT gene mutation AD Congenital, stable, circumscribed areas of amelanosis - leukoderma distribution: central anterior trunk, mid extremities, central forehead, midfrontal portion of the scalp Poliosis (white forelock in 80-90%) Absence of melanocytes within involved sites Absence of extracutaneous features (but do ophthalm, neuro and haring exams to exclude Waardenburg,

Answer 289

Several genes PAX3, MITF, SNAI2, SOX10 AD > AR achromia of the hair, skin, or both in the same pattern as piebaldism congenital deafness Heterochromia irides medial eyebrow hyperplasia (synophrys) dystopia canthorum (increased distance between the inner canthi, with a normal interpupillary distance) broad nasal root WS1 - classic WS2 - no dystopia canthorum WS3 - associated limb abnormalities - upper limb - hypoplasia, syndactyly Klein–Waardenburg syndrome WS4 - with Hirschsprung disease - Shah–Waardenburg syndrome, CNS dysfunction

Answer 290

Mostly AR diffuse pigmentary dilution of the skin, hair, and/or eyes number of epidermal and follicular melanocytes is normal partial or total absence of melanin pigment within melanocytes

Answer 291

AKs, lentigines SCC, BCC, melanoma Eyes - Amblyopia, reduced visual acuity, nystagmus, strabismus, photophobia (reduced melanin in RPE) Photoprotection FSE 6-12 months, self skin checks Ophthalm eye exam every 6-12 months Hearing test Emerging: Nitisinone (oral) - blocks tyrosine degradation

Answer 292

AR Gene: LYST Hyper and hypopigmentation in sun exposed sites Silvery hair Pigmentary dilution (Hair, skin, eyes) photophobia, nystagmus, and ocular hypopigmentation bleeding diathesis progressive neurologic dysfunction severe immunodeficiency (in immunodeficiencies and hypopigmentation chapters) Fatal without HSCT

Answer 293

AR pigmentary dilution of the skin and silvery-gray hair immunodeficiency neurologic impairment can get a hemophagocytic syndrome

Answer 294

BLOC1/2/3 AR pigmentary dilution of the skin, hair, and eyes Ocular manifestations of albinism - nystagmus and reduced visual acuity Bleeding tendency Interstitial pulmonary fibrosis Granulomatous colitis and cutaneous Crohns-like lesions recurrent bacterial infections

Answer 295

linear nevoid hypopigmentation - streaks and whorls along lines of Blaschko + extracutaneous: CNS - seizures, intellectual disability Hearing impairment Vision impairment Teeth MSK/orthopaedic

Answer 296

Cutibacterium acnes Orange-red on Woods lamp young women, darker skin, tropical climates poorly defined, nummular, non-scaly hypopigmented macules and small patches on the trunk topical 1% clindamycin 5% benzoyl peroxide nbUVB

Answer 297

most noticeable when there is surrounding vasodilation due to heat or emotional stress Diascopy - lesion becomes indistinguishable from surrounding skin, border disappears Accentuated with application of ice or heat

Answer 298

Selective allosteric inhibition of tyrosine kinase 2 TYK2 regulates signaling and functional responses downstream of IL12, IL23 and type 1 interferon receptors Deucravacitnib binds to the regulatory domain of TYK2 Think: I do crave a sit (deucravacitinib) so that I can regulate (regulatory) before saying hello the stairs (allosteric)

Answer 299

must not have achieved adequate response after at least 6 weeks of treatment with methotrexate prior to initiating treatment with this drug OR contraindication to methotrexate according to the Therapeutic Goods Administration (TGA) approved Product Information OR severe intolerance of, or toxicity due to, methotrexate AND significant interference with quality of life AND must not be undergoing concurrent PBS-subsidised treatment for psoriasis with each of: (i) a biological medicine, (ii) ciclosporin, (iii) apremilast Must be 18 years or older Must be treated by dermatologist, or being continued by a GP

Answer 300

B1 Lack of data to support its safety Case by case risk/benefit assessment

Answer 301

CIs: Hypersensitivity Infections, TB Malignancy (relative) Avoid use with live vaccines Rhabdomyolysis Ix: QFG LFTs Viral hepatitis screen Maybe lipids, CK

Answer 302

Nasopharyngitis, URTI HSV Mouth ulcers Folliculitis Acne Elevated CK Hypertriglyceridaemia Headache Nausea, diarrhoea No thromboembolic, cardiovascular events in trial

Answer 303

WATER BAD COPS Warfarin Antihistamines Anti epileptics Theophylline Retinoids BRAFi Benzodiazepines Antidepressants Dapsone Digoxin Cyclosporine Colchicine OCP Opiates Prednisolone Sonidegib Statin

Answer 304

JAKi Erythromycin Itraconazole, ketocanozole OCP Calcium channel blocker SSRIs Grapefruit

Answer 305

Rifampicin Isoniazid Dexamethasone Griseofulvin Epileptics (phenytoin, carbamazepine) St Johns Wort

Answer 306

Viruses - Hep C (esp oral LP), HHV6/7 Vaccines - Influenza, Hep B Contact allergy - Metals - amalgam (mercury), copper, and gold Autoimmune - thyroid, vitiligo, lichen sclerosus LP elsewhere: LPP scalp, nail LP, genital, cutaneous Drugs Smoking

Answer 307

Actinic Acute/exanthematous Annular Atrophic Bullous LP and LP pemphigoides (Difference between the 2 is that in LP pemphigoides there are circulating IgG autoantibodies against BP180) Hypertrophic Inverse LP pigmentosus Lichen planopilaris Linear LP (follows lines of Blaschko) Nail LP Oral LP Ulcerative Vulvovaginal Lichenoid drug eruption

Answer 308

ACE inhibitors Thiazides, diuretics Calcium channel blockers Beta blockers PD1, CTL4, 5FU Allopurinol NSAIDs HCQ Tetracyclines Anti TNF Anti epileptics Griseofulvin Statins Hydroxyurea Penacillamine Sulphonamides Gold

Answer 309

Lichenoid drug: Clinically: - Photodistributed/generalised (rather than wrists, lower legs, presacral - spares "classic" sites) - Uncommon to have mucosal involvement, LPP, nail LP - More papulosquamous or eczematous, less characteristic violaceous flat topped shiny polygonal papules etc - No Wickhams Histologically: - Parakeratosis (no parakeratosis in LP - think of the nuclei as little drugs) - Deeper perivascular infiltrate - Eos and plasma cells - Cytoid bodies in cornified, granular and upper spinous layers (whereas they are in lower spinous layer in LP)

Answer 310

Top CS Top CNI ILCS nbUVB HCQ Acitretin PO pred Apremilast MTX MMF AZA CsA TNF IVIG JAKi

Answer 311

Flat-topped pruritic violaceous papules and plaques polygonal-shaped Slightly shiny or transparent surface network of fine white lines called “Wickham striae” - corresponds histologically to thickened granular layer favouring the wrists, forearms, genitalia, distal lower extremities, and presacral area

Answer 312

Hyperkeratosis Focal parakeratosis Lichenoid pattern (+/- spongiosis) Perivascular and periadnexal/perieccrine infiltrate

Answer 313

Clinical: Multiple, tiny, discrete, shiny, skin-coloured papules Often arranged in larger clusters (Can be linear from Koebner) Favoured sites: flexor aspects of the upper extremities, the genitalia, and the anterior trunk Nails 10%, oral involvement rare Histo: dense well-circumscribed infiltrate composed of lymphocytes, histiocytes and epithelioid cells confined to the width of two to three dermal papillae closely apposed to the epidermis “clutched” by the surrounding hyperplastic rete ridges in a “ball and claw” configuration Overlying epidermis is atrophic and may have a parakeratotic cap Absence or thinning of granular layer

Answer 314

violaceous, keratotic, lichenoid papules arranged in a characteristic linear and reticulated pattern symmetrically distributed on the limbs and trunk greasy, crusted, scaly eruption/plaques involving upper/mid face seborrheic dermatitis or psoriasiform - like Chronic and progressive Treatment as for LP, symptomatic, not very successful Phototherapy, topical and systemic corticosteroids, methotrexate, cyclosporine, acitretin/retinoids

Answer 315

~10% of pts with LP have affected nails Mostly matrix changes: Thinning Longitudinal ridging Fissuring Pterygium

Answer 316

atrophic, bullous, erosive, papular, pigmented, plaque-like, and reticular - reticular most common chronic desquamative gingivitis Assess for oesophageal involvement - can develop dysphagia, strictures, stenosis, and even SCC

Answer 317

Triad of (Bolognia): (1) non-cicatricial loss of pubic and axillary hairs and disseminated spinous or acuminated follicular papules (2) typical cutaneous or mucosal LP (3) scarring alopecia of the scalp with or without atrophy OR triad of (DermNet): (1) Progressive patchy hair loss scalp (2) Non-scarring thinning of hair in the armpits and groin (3) Spiky rough bumps based on hair follicles

Answer 318

Lichenoid/interface pattern Dense, band-like lymphocytic infiltrate Colloid bodies representing apoptotic or dyskeratotic keratinocytes (also referred to as Civatte, hyaline, or cytoid bodies) Hyperkeratosis without parakeratosis (oral LP has para) focal increases in the granular cell layer irregular acanthosis with a “sawtooth” appearance “Max-Joseph spaces” - small separations between the epidermis and the dermis Shaggy band fibrinogen on DIF

Answer 319

Ready For a PIE and NAP Rheumatoid nodules Rheumatoid vasculitis + Bywaters Rheumatoid neutrophilic dermatitis Felty Palisaded neutrophilic & granulomatous dermatitis (PNGD) Interstitial granulomatous dermatitis (IGD) EED Neutrophilic dermatosis - Sweets APLS PG

Answer 320

In deep dermis or subcut Palisading granuloma around degenerated connective tissue Palisading macrophages around fibrinoid necrobiosis Histiocytes and lymphocytes

Answer 321

Sjogrens Chronic GVHD Primary systemic amyloidosis Sarcoidosis Hep C, HIV

Answer 322

lacrimal and salivary glands Xerostomia (risk dental caries, oral candidiasis) Xerophthalmia, keratoconjunctivitis (pain, photophobia, foreign body sensation) Arthritis - knees, ankles, chronic, progressive Cutaneous: Xerosis, petechiae, purpura (palpable and non-palpable), Raynauds, urticarial vasculitis, and annular erythema, nodular amyloidosis, Sweet syndrome, angular cheilitis Vaginal dryness Associated with RA, LE, DM, SSc

Answer 323

B-cell lymphomas, in particular extranodal marginal zone lymphomas of the MALT (mucosa-associated lymphoid tissue) type Peripheral neuropathy Interstitial pulmonary fibrosis Systemic vasculitis (kidneys) Dental caries, oral candidiasis Poorer prognosis: vasculitis, hypocomplementemia, cryoglobulinemia, parotid enlargement

Answer 324

Ocular: Artificial tears Steroid, NSAID, CsA eye drops Air humidifiers Ophthalm Oral: Sugar free gum Saliva substitute Neutral pH sodium fluoride gum Dental/oral medicine (Avoid anticholinergics) Cutaneous: TOP CS Annular erythema - HCQ, pred Vasculitis (if severe) - other immunosuppressants, rituximab, cyclophosphamide

Answer 325

Acrokeratosis paraneoplastica Acral psoriasiform plaques Violaceous Involvement nose and helices Ridging (horizontal or longitudinal) of nails Malignancy - most commonly in the upper aerodigestive tract (pharynx, larynx or esophagus) Upper respiratory tract or GI

Answer 326

short episodes at the start of the illness, but prolongation of the episodes as the condition progresses Flushing and erythema of the head and neck Vascular rosacea-like changes Pellagra-like dermatitis and sclerodermoid changes Oedema and induration (face > extremities) - leonine facies Pigmentation Diarrhoea Wheezing/bronchospasm Peptic ulcers Right sided cardiac dysfunction Hypotension Tachycardia Malignancy - mid gut tumours with liver mets (small intestine, appendix, proximal colon) - minutes, erythema pallor and cyanotic blue - gastric and bronchial carcinoid tumours - longer intense red, purple flush Release of peptides and amines (serotonin, histamine) from neuroendocrine tumour (NET) in the gut (most common) or pancreas, kidneys, ovaries, testes NET can occur in MEN1 and Peutz Jeghers Diagnosed 24 hr urine 5-HIAA (hydroxyindoleacetic acid)

Answer 327

fine lanugo hairs in a generalized distribution or localized to the face most often carcinoma of the lung, colon or breast Colon > lung > breast Often poor prognosis metastatic also associated with AN, tongue papillae hypertrophy, altered taste and smell

Answer 328

adenopathy, extensive skin patch overlying (a) plasmacytoma large red to violet–brown patch

Answer 329

FOAMED DS Familial Obesity Autoimmune (Sjogrens, SSc, lupus) Malignancy (esp GI/gastric) (esp if tripe palms and oral involvement) Endocrine (hypothyroidism, Addisons, hyperandrogenism, acromegaly, Cushings) Diabetes Drugs Syndromes (Cruzon, HAIRAN)

Answer 330

MIXED CANVAS Mucinoses - scleromyxoedema (IgG paraproteinaemia), sclerodema Immunobullous - LABD, IgA pemphigus, Sneddon Wilkinson, EBA Ichthyosis Xanthoma - plane and NXG (IgG paraproteinaemia) EED Dermatomyositis Cryoglobulinaemia Amyloidosis (primary systemic) - plasma cell dyscrasia > multiple myeloma Neutrophilic dermatoses Vasculitis Acanthosis nigricans Schnitzler syndrome (IgM paraproteinaemia) + POEMS

Answer 331

COPING Corticosteroids OCP Protease inhibitors Insulin Nicotinic acid Growth hormone

Answer 332

Chronic urticarial lesions Histologically neutrophil rich Fevers, arthralgias, bone pain associated with an IgM, usually κ, paraproteinemia (less common IgG); lymphoplasmacytic malignancies develop in ~15% of patients Responds well to IL1 anakinra or canakinumab

Answer 333

Polyneuropathy, Organomegaly, Endocrinopathy, M-protein (Monoclonal gammopathy) and Skin changes Skin changes: Glomeruloid hemangioma - pathognomonic (but minority of patients) Cherry angiomas Hyperpigmentation Hypertrichosis Sclerodermoid induration Acrocyanosis Acquired facial lipoatrophy Hyperhidrosis Digital clubbing Plethora (swelling and puffiness) Leukonychia Malignancies: Osteosclerotic myeloma Castleman disease Plasmacytomas Other associations/signs: Peripheral oedema, ascites, pulmonary effusions, papilledema, thrombocytosis, polycythemia, and increased serum levels of VEGF

Answer 334

Liaise with GP History - symptoms (LOW, B symptoms, blood loss, lumps), Fam Hx cancer, smoker/ETOH Exam - FSE, LN, liver/spleen Investigations - Check up to date with age appropriate screen (mammogram/breast USS, pap smear, FOBT, PSA) - Bloods - FBE, UEC, LFTs, CA-125 (ovarian), CEA (colon), AFP (testicular, liver), CA-19 (pancreas), B2M (lymphoma, myeloma, leukaemia) - FOBT x3 - Urine (cytology, blood) - Imaging - CXR, abdo/liver/renal USS, pelvic USS, CT CAP - Referral for gastroscopy/colonoscopy

Answer 335

IgA monoclonal gammopathy or multiple myeloma SAPHO PG RA Lupus Thyroid

Answer 336

AR EVER1 or EVER2 Red brown patches/plaques due to HPV with high risk SCC transformation

Answer 337

GRANDE CLUBS Grannuloma annulare (disseminated) Acquired perforating dermatosis AN Necrobiosis lipoidica Diabetic dermopathy Eruptive xanthomas Cheiroarthropathy Candida Lipodystrophy Lower limb ulcer, PVD Bullae Scleredema

Answer 338

Hyperthyroid Pruritus Urticaria Increased sweating Hyperpigmentation Pretibial myxoedema Thyroid acropachy (acral soft tissue swelling, periostitis, and clubbing of the digits) Vitiligo Alopecia Clubbing Onycholysis, koilonychia Hypothyroid Acquired ichthyosis and palmoplantar keratoderma Eruptive and/or tuberous xanthomas Vitiligo Alopecia Thin, brittle, striated nails

Answer 339

Altered subcutaneous fat distribution Rounded “moon” facies Dorsal cervical fat deposition “buffalo hump” Pelvic girdle fat deposition Reduced fat in the arms and legs Skin atrophy Multiple striae Fragile skin Prolonged wound healing Purpura from minor trauma Cutaneous infections Pityriasis versicolor Dermatophytosis and onychomycosis Candidiasis Acne Hirsutism

Answer 340

Juvenile polyposis and HHT

Answer 341

FAT FACES PALMS Fissured tongue Anetoderma Tinea Folliculitis AA Cutis laxa, calcinosis (milia like) EPS, eczema Syringomas, seb derm Psoriasis, PRP, palmar crease Acrocyanosis Lichen nitidus, livedoid plaques Melanoma Suppurativa, hidradenitis

Answer 342

Can be systemic or benign (skin only) Associated with SLE, DM, APLS Early lesions – pale red papules with central necrosis Late lesions – atrophic ivory scars Small infarctions in the GI mucosa. Haemorrhage and intestinal perforation CNS involvement, pleuritis, pericarditis

Answer 343

Circumscribed areas of lentiginous hypermelanosis, alopecia, generalized nail thinning Adenomatous GI polyps, diarrhea, abdominal pain Weight loss due to malabsorption

Answer 344

Spider angiomas and other telangiectasias Palmar erythema Dilated abdominal wall veins Terry nails (white nail bed) Muehrcke nails (transverse white bands) Sparse axillary, pubic and pectoral hair Pruritus Jaundice

Answer 345

Blue lunulae Pretibial hyperpigmentation Kayser–Fleischer ring

Answer 346

Hep C Zinc deficiency Coeliac Crohns

Answer 347

AD RASopathy BRAF > MAP2K1, MAP2K2 > KRAS Skin: Generalized ichthyosis-like scaling, keratosis pilaris, café-au-lait macules, sparse curly hair, sparse or absent eyebrows and eyelashes CVS: Pulmonic stenosis, atrial septal defects, hypertrophic cardiomyopathy characteristic facies, short stature and developmental delay

Answer 348

AD RASopathy HRAS > KRAS Skin: Lax skin on the hands and feet, deep palmoplantar creases, periorificial papillomas, acanthosis nigricans, curly hair CVS: Pulmonic stenosis, hypertrophic cardiomyopathy, arrhythmias Increased risk of rhabdomyosarcoma and bladder carcinoma Mental and growth delay

Answer 349

CBS, AR Marfanoid habitus, lens subluxations (downwards), venous and arterial thrombosis, atherosclerosis, intellectual impairment Malar flush, livedo reticularis, diffuse pigmentary dilution, tissue-paper like scars

Answer 350

AD TGFBR1 and 2 Translucent skin, easy bruising, atrophic scarring (Atrophoderma Vermiculatum) Aortic aneurysms, arterial tortuosity; significant risk of aortic dissection and rupture

Answer 351

AD FBN1/Fibrillin 1 Tall stature with long limbs, arachnodactyly, dolichocephaly, myopia and ectopia lentis Striae Decreased subcutaneous fat Elastosis perforans serpiginosa Dilation and dissection of the ascending aorta, mitral valve prolapse and regurgitation

Answer 352

Thick yellow nails, disappearance of cuticle lymphedema respiratory tract involvement, including chronic bronchitis, bronchiectasis, sinusitis, and pleural effusions Treatment: vitamin E 1200 IU/day pulse itraconazole (200 mg twice daily for 1 week a month) or fluconazole (150 mg daily for 1 week a month)

Answer 353

EBV driven B cell proliferative disease Prominent angiocentricity Skin involvement panniculitis, indurated plaques, erythematous dermal/subcutaneous papules and nodules Lungs - cough, SOB, chest pain Fever, arthralgias, myalgias

Answer 354

Pale color, sallowness Xerosis or acquired ichthyosis Pruritus Acquired perforating dermatosis Pseudoporphyria Calciphylaxis Uremic frost

Answer 355

Multiple cutaneous leiomyomas Predisposition to develop aggressive renal carcinoma Uterine leiomyomas/fibromas mutations in the gene encoding fumarate hydratase

Answer 356

Epithelioid atypical Spitz tumors, cutaneous melanoma Uveal melanoma Mesothelioma Clear cell renal cell carcinoma AD inheritance

Answer 357

Congenital - Cutis marmorata telangiectatica congenita Vasospasm - Cutis marmorata/physiologic livedo reticularis - Primary (idiopathic) livedo reticularis - Autoimmune connective tissue diseases (e.g. SLE) - Raynaud phenomenon/disease Vessel wall pathology - Vasculitis --- Cutaneous polyarteritis nodosa --- Systemic polyarteritis nodosa --- Cryoglobulinemic vasculitis --- ANCA-associated vasculitides --- Autoimmune connective tissue disease-associated vasculitis (e.g. rheumatoid arthritis, SLE, Sjögren syndrome) - Lymphocytic thrombophilic arteritis - Calciphylaxis - Sneddon syndrome - Deficiency of adenosine deaminase 2 (DAD2) - Livedoid vasculopathy (also intraluminal obstruction) Intraluminal pathology - Increased normal blood components --- Thrombocythemia --- Polycythemia vera - Abnormal proteins --- Cryoglobulinaemia --- Cryofibrinogenaemia --- Cold agglutinins --- Paraproteinaemia - Hypercoagulability --- Antiphospholipid syndrome --- Protein S and C deficiencies --- Antithrombin III deficiency --- Factor V Leiden mutation --- Homocystinuria, hyperhomocysteinemia --- Disseminated intravascular coagulation - Thrombotic thrombocytopenic purpura - Embolic --- Cholesterol, septic emboli --- Atrial myxoma --- Nitrogen (decompression sickness) --- Carbon dioxide arteriography - Thromboses within vessels (e.g. APS, heparin or warfarin necrosis) - Hyperoxaluria (intracellular crystal deposition) - Intralymphatic histiocytosis Other - Medications amantadine, norepinephrine, interferon - Infections hepatitis C [vasculitis], Mycoplasma spp. [cold agglutinins], syphilis - Neoplasms e.g. pheochromocytoma - Neurologic (via vasospasm or vasodilation) - reflex sympathetic dystrophy, complex regional pain syndrome

Answer 358

AR autoinflammatory disorder Features of Sneddon syndrome and PAN (vasculopathy and vasculitis) intermittent fevers early-onset lacunar strokes hepatosplenomegaly hypogammaglobulinemia and lymphopenia cutaneous nodules and livedo racemosa

Answer 359

JAK2 Aquagenic pruritus Livedo racemosa/reticularis Erythromelalgia Raynauds Superficial thrombophlebitis Early satiety, abdo pain, splenomegaly, fatigue, headache, dizziness, hearing and visual disturbance Aspirin, venesection, ruxolitnib

Answer 360

Rosacea Menopause Anxiety Migraine Autonomic dysfunction Frey syndrome Mastocytosis Carcinoid Phaeochromocytoma POEMs Thyrotoxicosis Medullary carcinoma of thyroid Pancreatic tumours (VIPomas) Prostaglandin-secreting renal cell carcinoma Exogenous - alcohol, prostaglandins, ACEi, calcium channel blockers, CsA, hydralazine, nicotinic acid, opioids, nitrates eg GTN, sildenafil, tamoxifen Spoiled scombroid fish, MSG or sulfites in foods AICTD

Answer 361

FBE TFTs Hormones - oestrogen, FSH, LH, AMH Serum tryptase (masto) Plasma free metanephrines (phaeochromocytoma) Serum chromogranin A levels (carcinoid) 24 hour urine for 5-HIAA (carcinoid) 24 hour urine for fractionated metanephrines (phaeochromocytoma) 24 hour urine for histamine metabolites (masto) CT/MRI Octreotide/somatostatin receptor scintigraphy to localise the carcinoid tumour ANA, ENA, dsDNA, C3/4 (for lupus) Biopsy if rash

Answer 362

type 1 – associated with thrombocythemia (increased number of platelets/abnormalities in platelet function) - more often unilateral type 2 – primary or idiopathic form type 3 – associated with underlying causes other than thrombocythemia

Answer 363

SCN9A AD 5% fam hx Often de novo

Answer 364

myelodysplastic syndromes, diabetes mellitus, peripheral arterial disease, vasculitis, systemic lupus erythematosus, and other autoimmune CTDs incl morphoea and SSc (often with Raynauds), infection (HIV), gout therefore Ix include: HIV, ANA, RF, uric acid levels

Answer 365

MDT - derm, pain, neurology, haem Educate, avoid triggers Elevation, avoid prolonged dependency Cooling: fans, wet dressings, and ice packs wrapped in towels (safe cooling techniques) To treat vascular dysfunction: Aspirin CCBs (diltiazem) - may exacerbate Pentoxyfilline Vitamin C, vitamin E Pain: Lidocaine 5% patches to area compounded gels 1% amitriptyline/0.5% ketamine or midodrine 0.2%, applied a median of 5 times per day Menthol Gabapentin, pregabalin Amitriptyline Fluoxetine (SSRIs) Pain clinic CBD oil, opioids Other: sodium channel blocking agents (e.g. mexiletine, flecainide) - especially primary inherited Carbamazepine Prostaglandins - misoprostol Iloprost TOP: 10% capsaicin cream 1% amitriptyline/0.5% ketamine gel

Answer 366

SUGAR PATCH Spider naevi (can be a/w liver dz, oestrogen excess) Unilateral naevoid telangiectasia Generalised essential telangiectasia Ataxia telangiectasia Angioma serpiginosum Radiotherapy and rosacea Poikiloderma - XP, Rothmunnd Thomson, DKC, GVHD Atrophies from steroid TMEP CREST CCV - cutaneous collagenous vasculopathy HHT, CMAVM2

Answer 367

Pregnancy Cryoglobulinaemia

Answer 368

Chronic vs acute Spontaneous vs inducible (usually chronic) Acute (often triggered by infection/medication) Chronic spontaneous urticaria Inducible urticaria including cholinergic - Temperature changes (cold urticaria, heat urticaria) - Exercise induced - Contact (symptomatic dermographism, delayed pressure urticaria) - Mechanical (vibratory agioedema) - Solar - Aquagenic

Answer 369

Chronic urticaria - 2-3% Lifetime prevalence of urticaria 8-22% Prognosis 50% clear within 6 months tto 5 years But 20% still have 10 years later And 10% 20 years later

Answer 370

Often inherited Can be acquired (e.g. B-cell lymphoproliferative disorders, plasma cell dyscrasias) Recurrent angioedema without wheals laryngeal oedema colicky abdominal pain Trauma and oestrogen can trigger attacks due to bradykinin formation rather than release of mast cell mediators

Answer 371

AD Type I - reduced levels of C1 esterase inhibitor (C1 inh) Type II - reduced C1 inh function Type III - normal C1 inh activity - later age onset (teens)

Answer 372

mast cell is the primary effector cell of urticaria Release of chemical mediators histamine and bradykinin from mast cells Circulating autoantibodies against IgE or IgE receptor on mast cells - causes activation and degranulation of mast cells

Answer 373

Urticarial vasculitis Schnitzler Cryopyrin associated periodic syndrome (e.g. Muckle-Wells syndrome)

Answer 374

autoimmune thyroid disease other autoimmune conditions, including vitiligo, insulin-dependent diabetes, rheumatoid arthritis, and pernicious anaemia ?H Pylori ?Strongyloidiasis ?Haem malignancies ?dental infections

Answer 375

upper dermal oedema with a mild mixed perivascular infiltrate of lymphocytes, eosinophils, basophils, and some neutrophils

Answer 376

Thyroid function and antibodies FBE, ESR, CRP IgE (higher IgE suggests responsiveness to omalizumab, normal IgE indicates responsiveness to CsA) - in treatment resistant cases Helicobacter pylori (not routine, but if symptoms) Coeliac serology (if symptomatic) If inducible: Solar - ANA, porphyrins Aquagenic - annual FBE (polycythaemia rubra vera) Cold - ?cryoglobulins Angio-oedema without weals - C3, C4 If low C4, then C1 esterase inhibitor level and function If fevers/raised inflammatory markers/arthralgias/systemically unwell: If susp for autoinflammatory syndrome then can add serum amyloid A SPEP if concern for Schnitzler Genetic test if suspecting CAPS

Answer 377

leukotriene receptor antagonist Especially handy for urticaria exacerbated by salicylates and where angio-oedema is predominant Neuropsychiatric SEs especially in children (nightmares/terrors, aggression, behaviour change, stuttering Preg Cat B1

Answer 378

tricyclic antidepressant with very potent H1 and H2 antihistaminic properties significant sedative and anticholinergic properties

Answer 379

Cyproheptadine Promethazine Chlorphenamine Hydroxyzine Diphenhydramine Lipophilic and readily cross the blood brain barrier Longer half life in aging patients due to higher percentage adipose composition

Answer 380

General measures Avoid triggers - stop NSAIDs, ACEi, inducible urticaria triggers DLQI, UAS7, HADS (hospital anxiety and depression score) Educational materials Topicals Menthol emollient Second generation H1 antihistamine 1 daily (regular daily licensed dose). If inadequately controlled, then increase up to fourfold. (Following complete symptom control, attempt stepwise reduction). Try a different second gen H1 antihistamine if one not effective. Progression of therapy every 2-4 weeks Add montelukast 10mg nocte. If associated GORD, then can add H2 antihistamine (famotidine). Oral pred rescue treatment 0.5mg/kg few days (short, infrequent) Second line: (Add) Omalizumab or CsA (for 3-6 months) Third line: AZA Dapsone Doxepin HCQ IVIG MTX MMF nbUVB (30 treatments, repeated after 12 months) Sulfasalazine Oral tacro Tranexamic acid (if predominantly angio-oedema)

Answer 381

Blood test in CSU If +ve, higher responsiveness to CsA If -ve, higher responsiveness to omalizumab

Answer 382

Same as for CSU re: antihistamines Omalizumab can be 2nd line option for all (except maybe VA) Epipen if risk of anaphylaxis (esp cold or cholinergic) Montelukast in some types (delayed pressure) Cholinergic urticaria - Anticholinergics (oxybutynin), beta blockers (propranolol), danazol, phototherapy Cold urticaria - CsA Delayed pressure urticaria - Dapsone or sulfasalazine Solar - sunprotection - UV prophylactic phototherapy/hardening (following photoinvestigation for wavelength) Symptomatic dermographism - nbUVB

Answer 383

Muckle Wells Familial cold autoinflammatory syndrome Neonatal onset multisystem inflammatory disorder

Answer 384

Fragmentation of leucocytes with nuclear debris (leucocytoclasia), endothelial swelling or damage, red cell extravasation, rarely fibrin deposition

Answer 385

Cetirizine Loratadine Fexofenadine Desloratidine Levoceterizine Mizolastine (QT prolongation) Acrivastine

Answer 386

Confusion Drowsiness Balance problems Urinary retention (ask re: prostate hyperplasia) Bladder infection Glaucoma (risk acute angle glaucoma, esp if other annticholinergics) Increased risk fall and fractures Prolongation QT interval, tachycardia Constipation

Answer 387

Most inhibit cytochrome P450, esp isoenzyme CYP2D6 e.g. Diphenhydramine Therefore increase serum levels of med metabolised by CYP2D6 Beta blockers Antidepressants Tramadol (Note: second gen H1 antihistamines DO NOT inhibit or induce 3A4 or 2D6) Other interactions: TCAs, benzos, quetiapine, tramadol, dementia meds, SSRIs

Answer 388

Flucloxacillin - 2C9 Terbinafine - 2D6 Itraconazole - 3A4

Answer 389

Typically 5-15 minutes (can be delay 1 hr) UVA most common, can be combo

Answer 390

In ~30% AD ADGRE2

Answer 391

ISRs GI Headache Fatigue Arthralgia Infections - URTI, nasopharyngitis, sinusitis Dizziness Worsening urticaria Anaphylaxis <0.1% in asthma patients

Answer 392

IV C1 inh concentrate or recombinant C1 inh ecallantide (a kallikrein inhibitor) icatibant (a specific bradykinin B2 receptor antagonist) FFP Tranexamic acid or C1 inh concentrate pre-op

Answer 393

Episodic angioedema with eosinophilia weight gain, and fever increased IL5

Answer 394

episodic massive plasma exudation from blood vessels, leading to potentially life-threatening hypotension, analogous to anaphylaxis May have angioedema associated with an IgG paraproteinemia

Answer 395

0.7% cantharadin Cantharadin plus = cantharadin 1%, podophyllin 5%, sal acid 30% Indications: Verruca Molluscum contagiosum CI: Hypersensitivity Mucous membranes Diagnostic uncertainty Caution: diabetes, PVD SEs: Pain/discomfort (from blister) - pain not common PIH Annular wart formation Infection/cellulitis

Answer 396

In office treatment If wart, pare down Cantharadin accurately applied (wooden end of cotton applicator) Dry, then micropore tape over the top, keep on for 4-6 hours Then remove and wash with soap and water Blister will form 24-48 hours Over few days blister will dry and come off Healing can take 7 days May require repeat treatment (esp if wart, 1-3 week intervals) No scarring

Answer 397

Antifibrinolytic Melasma Hereditary angio-oedema 250mg BD for 3 month course Or 500, max 750mg daily (split dosing)

Answer 398

CI Hypersensitivity Personal or fam hx of coagulopathy Active or hx of: - Thromboembolic disease - Cardiovascular disease, stroke - Anticoagulants - Hypercoagulable comorbidities (malignancy, renal dysfunction) Pregnancy (Cat B1) Lactation OCP Caution if smoking SEs: GI - abdo pain, N+V Menstrual irregularities Headache Back pain Recurrence on cessation Disturbance colour vision?

Answer 399

Small molecule inhibitor of phosphodiesterase 4, preventing the degradation of cAMP to AMP, therefore suppressing production of TNF-α, IL-17, and interferon (IFN)-γ CPP Nail and scalp PsO Behcets Aphthous stomatitis Sarcoidosis LP CYP450 metabolism - interactions (esp inducers, reduces levels of apremilast and efficacy) Pregnancy Category B3

Answer 400

Precautions: ·Seizure disorder or manic-depressive disorder – lower seizure threshold, manic episode Cardiac arrythmias Side effects: CNS: Sedation, lower seizure threshold Cardiac: QT prolongation, dysrhythmia, hypotension Weight gain Anti-cholinergic – dry mouth, blurry vision, urinary retention, constipation Discontinuation symptoms – headache, dizziness, insomnia, nausea, REM rebound vivid dreams

Answer 401

CIs: Hypersensitivity Pregnancy, lactation Severe depression CRF eGFR <30 Children SEs: GI (25%, usually in first week) - diarrhoea, nausea Weight loss Headache Mood/depression URTI, nasopharyngitis Fatigue Back pain

Answer 402

50% improve by half 33% improve by 3/4 10% improve by 90%

Answer 403

MoA: anti-neutrophilic, anti-inflammatory, immunosuppressive Suppresses the generation of inflammatory oxygen intermediates Indications: EN EI nodular vasculitis subacute nodular migratory panniculitis Neutrophilic dermatosis sporotrichosis Preg Cat D Interactions: Lithium Amiodarone

Answer 404

Haem - cytopaenias Iododerm/halogenoderma, acneiform, vegetative plaques, dermatitis, vasculitis; flares DH and pustular psoriasis CNS: headache/cold-like symptoms, brassy taste, burning mouth; depression Abdomen: pain, diarrhoea, anorexia Thyroid - hypothyroidism, thyrotoxicosis "HI CAT" Haem - cytopaenias (site the proposal, engagement) Iododerm/halogenoderma, acneiform, vegetative plaques, dermatitis, vasculitis; flares DH and pustular psoriasis (let go skin) CNS - headache and mood disturbance (with pregnancy) and brassy/metallic taste (from the bling) Abdo Tying down (hypothyroid)

Answer 405

Hypersensitivity Hypothyroidism Renal disorder Cardiac disease Addison’s Hyperkalaemia Pregnancy (Cat D) (gumpy GF 'K' : grievances ADDING up, dehydrated, heartbroken, depressed/gaining weight = hypothyroidism)

Answer 406

Drops in orange juice 1 drop = 50mg Start 100mg daily Up to 3 drops TDS (150mg TDS)

Answer 407

Baseline: FBC, EUC, LFT, TFTs, thyroid antibodies Follow up: FBC, TFTs 1 month; then 6monthly

Answer 408

Calcineurin inhibitor Target: T cells Inhibits IL2 Start at 2-3mg/kg/day (divided dose) Max 5mg/kg/day (divided dose) Comes in 25mg and 100mg capsules Max 12 months Preg Cat C

Answer 409

Hypersensitivity Uncontrolled hypertension Renal impairment (significant) Malignancy - including CTCL Infection (relative) Controlled HTN (relative) Concomitant immunosuppressive agents or medications that interact Concurrent phototherapy Inability to meet monitoring requirements Epilepsy (lowers seizure threshhold) Pregnancy, lactation (relative) - Category C (discussion of risks + benefits), excreted in breast milk (not recommended) - IUGR, PE/HTN

Answer 410

SIR HASN'T GB SCCs Infection Renal, HTN Hyperlipidaemia, hyperuricaemia, hyperK, hypoMg, hypertrichosis Acne Sebaceous hyperplasia Neuro: paraesthesia Tremors Gum hyperplasia, GI Breast lumps, benign fibroadenomas

Answer 411

Recheck BP 2 weeks later If remains elevated then can reduce dose by 25-50% (However, some studies have suggested the HTN may not be dose related) Other option is to add anti-HTN - amlodipine

Answer 412

If Cr >25-30% - repeat in 2 weeks If still >30% - cease OR reduce dose by at least 1mg/kg, repeat bloods 1 month If still >30% - cease CsA

Answer 413

CYP450 inhibitors increase CsA levels CYP450 inducers decrease CsA levels Nephrotoxic - NSAIDs, ACEi Increased risk pseudotumour cerebri - Tetracyclines

Answer 414

Baseline BP 2 separate measurements Urinalysis (PCR, ACR) Hx and Ex to rule out active infection and malignancy Fasting lipids FBE, UEC, CMP, LFTs, betaHCG Pre-immunosuppression screen, vaccinations up to date, age appropriate malignancy screen Consider baseline uric acid (if high risk/hx gout) Monitoring 2 weekly f/u for 2 months - checking BP and UECs Monthly FBE, UEC, CMP, LFTs, lipids +/- uric acid

Answer 415

Active vs T and B cells Purine analogue Pro-drug of 6 mercaptopurine

Answer 416

AZAAA like "howzaat?" (cricket term) Fat Batter Has a Mediocre HIT at the 'G Fatigue Bone marrow suppression Hypersensitivity syndrome Malignancy Hepatotoxic Infection Teratogen GI

Answer 417

Previous alkylating agent - cyclophosphamide, chlorambucil, melphalan 6-MP sensitivity/hypersensitivity to AZA Live vaccines Pregnancy (category D) Lactation Uncontrolled infection Active malignancy Relative: Concomitant use of allopurinol Concomitant use of other myelosuppressant (MTX) Renal, hepatic issues Malignancy

Answer 418

Allopurinol - blocks xanthine oxidase pathway for AZA - can result in toxicity Other immunosuppressants/myelosuppressants (MTX, penicillamine) Other TPMT inhibitors sulfasalazine, mesalazine Decreases levels of warfarin and CsA Increased myelosuppression with: allopurinol, ACEi, MTX

Answer 419

Start 1mg/kg/day (often 50mg) max 2-2.5mg/kg/day 6-12 month course TPMT NUDT15 Pre-immunosuppression Age appropriate malignancy screen betaHCG Low TPMT = do not use. Medium TPMT = max 1mg/kg/day TPMT 90% normal, 10% intermediate, <1% low FBC, UEC, LFT – 2weekly for 2 months; then every 2-3 months when dose stable More frequent in patients with hepatic, renal disease or high dose FSE and LN–6 monthly

Answer 420

Folate antagonist inhibits DNA synthesis, inhibition of cell division Category D Females: Spontaneous abortion, teratogenic, but some studies show no risk of congenital abnormalitie Males: Oligospermia Wait 6 months for both Psoriasis, MF, Sezary, AD, PRP, PLEVA, immunobullous, AI-CTD, vasculitis, neutrophilic dermatoses, morphea

Answer 421

Absolute: Hypersensitivity, Pregnancy, Lactation Relative: Renal disease Liver disease – abnormal LFTs, hepatitis, NASH Excessive ETOH DM, obesity Severe haematologic abnormalities Active infection Immunodeficiency syndrome Unreliable patient

Answer 422

Dose: cumulative, incremental, frequency. Age > 60 years (risk of reduced renal clearance). Alcohol consumption > 15 g/day. Obesity, type 2 diabetes, NASH, hypercholesterolaemia Pre-existing liver disease (e.g. hepatitis B and C). Renal failure (decreased clearance). Liver disease is more common in psoriasis than in rheumatoid arthritis. Other drugs: vitamin A, non-steroidal anti-inﬂammatory drugs. No folate FIB-4 score (fibrosis 4) Fibroscan - high negative predictive value (limited in obese) More historical: P3NP (procollagen type III N-terminal peptide) - high negative predictive value

Answer 423

Dosing 10-25mg weekly PO Paediatric: 0.4-0.6mg/kg/week; start ½ dose for 2 weeks Monitoring FBC – Week 2 and 4 FBC, EUC, LFT – 3 months, then 6 monthly Non fasting lipids, HbA1c every 6-12 months BMI, WC every 6-12 months Consider fibroscan delayed baseline; then 1-3 yearly Bloods taken 5-6 days after dose – Weekend or Monday before dose

Answer 424

Drug interactions (trimethoprim, sulfmethoxazole, NSAIDs) Renal impairment Elderly No folate Daily dosing Low albumin Major illness Can treat with folinic acid (leucovorin)

Answer 425

Anti-inflammatory, anti-mitotic Indications: Neutrophilic CSVV Aphthous stomatitis Neutrophil-rich autoimmune blistering dermatoses - EBA, LABD, DH FMF CIs: Absolute: hypersensitivity, blood dyscrasia Relative: Serious GI, renal, hepatic disorders Pregnancy, lactation Drug interactions: CYP450 3A4 metabolised Preg Cat D

Answer 426

Drop of your BAG TO the clerk B12 malabsorption Alopecia (anagen effluvium) GI: nausea, abdominal pain, diarrhoea, cramping Toxicity: dehydration, hyponatremia, hypokalemia, renal failure, liver failure, respiratory distress, cardiac failure, arrythmia, bone marrow toxicity cytopenias, DIC Oligospermia NGV - discussing nausea, diarrhoea; it's a gallery not a museum so there are no whales, maybe just some little ones in the odd painting (oligospermia); think of a bald artist lady; year 12 VCE exhibit (B12 malabsorption); Toxicity - art addict not eating or drinking, maybe just wine, they love the art so much they end up in resp distress and cardiac failure and arrythmia, they like art that is very out there (DIC), and they can never get enough of a sight (cytopaenias, BM toxicity)

Answer 427

0.5mg daily increasing BD or TDS FBC, UEC, LFTs monthly for 3 months then q3 months

Answer 428

Peripheral keratin colarette Glomerular vessels, uniformly distributed

Answer 429

Yellow homogenous area With linear and telangiectatic vessels

Answer 430

Bluish to opaque homogenous areas May have linear and telangiectatic vessels

Answer 431

Yellow/cream colour, branching linear vessels, +/- shiny white structures

Answer 432

Erythema, haemorrhage, whitish structures, irregular vessels

Answer 433

Linear branching crown vessels, over yellow homogenous structureless areas Or cluster of yellow/white sebaceous gland aggregates, each with a central comedo-like opening, and small peripheral crown vessels (Sebaceous adenoma larger)

Answer 434

Syringocystadenoma papilliferum Trichoepithelioma/trichoblastoma BCC Tricholemmoma desmoplastic tricholemmoma Sebaceous adenoma Apocrine adenoma Poroma

Answer 435

Yellow sebaceous gland aggregates outlined by crown vessels, surrounding erythema

Answer 436

White sclerosis, linear branching vessels, ?sebaceous gland aggregates with crown vessels

Answer 437

Short curvilinear vessels Perifollicular hypopigmentation (indicating scarring) Perifollicular hypopigmentation might coalesce

Answer 438

Central keratin/scale With whitish halo/surrounding whitish homogenous area Radial linear vessels Peripheral erythema

Answer 439

Pink structureless area Poorly focused linear branching vessels Shiny white structures

Answer 440

Yellow-orange background, haemorrhage, polymorphous vessels

Answer 441

WNT10A AR Hidrocystomas PPKD Hypodontia Nail dystrophy Hypotrichosis Facial telangiectatic erythema Eccrine syringofibroadenomas SCC

Answer 442

FLCN gene AD Fibrofolliculomas Trichodiscomas Lung cysts and pneumothorax Renal Ca Angiofibromas Connective tissue naevi ?Melanoma

Answer 443

Gene: CYLD Cylindromas Spiradenomas BCCs Trichoepitheliomas Parotid/salivary lesions

Answer 444

Papule or nodule central follicular ostium or punctum with a small tuft of lightly pigmented hairs Histo: Central cystic space central orthokeratin Relatively well-developed and occasionally oddly formed vellus follicles protrude in radial fashion from the central structure enveloped by a vascularized fibrous (angiofibroma-like) stroma

Answer 445

Strands of basaloid cells emanate from folliculosebaceous unit, forming a mitt-like configuration encasing delicate fibrous stroma

Answer 446

postzygotic somatic mutations in HRAS (95%) and KRAS Schimmelpenning syndrome (ocular, CNS, skeletal) or phakomatosis pigmentokeratotica (combined with speckled lenntiginous naevus - consider if extensive

Answer 447

Epidermal hyperplasia Papillomatosis Many normal or enlarged sebaceous glands, usually unassociated with mature hair shafts Many apocrine glands

Answer 448

Well circumscribed dermal nodular tumour Epithelial islands small to medium sweat ductal structures, eccrine or apocrine Or anastamosing trabeculae of basaloid cells Mucinous/myxoid stroma Sometimes hyalinised areas in stroma Relevant negatives: No connection to epidermis No retraction artefact/clefting or stromal mucin No atypia or mitoses

Answer 449

Circumscribed basaloid tumour islands Often in reticulated or cribriform pattern (interconnecting cords) Horn cysts common Peripheral palisading of nuclei, butt no clefting/artifactual retraction between tumour and stroma Loose stroma with fibroblasts surrounds basaloid islands Papillary mesenchymal bodies CK20 stain favours trichoepithelioma over BCC

Answer 450

firm skin-coloured to erythematous annular plaque with a central dell usually solitary and on the cheek Histo: Similar to trichoepithelioma - thin strands of basaloid cells; sclerotic stroma

Answer 451

solitary skin-coloured or bluish nodule childhood/adolescence head and neck rock hard tent sign - angulated shape when stretch overlying skin Associations: CTNNB1 gene Turner syndrome Myotonic dystrophy Gardner syndrome Histo: Circumscribed nodule resembling a cyst Lining - epithelial, some basaloid cells Shadow/ghost cells (pale empty space where nucleus used to be) Shards of keratinous debris, calcification, ossification Foreign body multinucleated giant cells and granulmatous inflammation

Answer 452

Hyperkeratosis with downward lobular growth of epidermis Clear cells (keratinocytes) - glycogen within cells, PAS pos Thin rim of basal cells palisade at edge of lobule clear cells Thick basement membrane Outer root sheath differentiation can be demonstrated with CD34 Loss of PTEN expression via IHC is suggestive of Cowden's

Answer 453

Sebaceous adenoma - Circumscribed, lobular tumour of sebocytes and basaloid cells - > 50% of cells are mature sebaceous cells/ sebocytes - Basaloid cells peripherally - Mitoses but not cytological atypia Sebaceoma (think: basic/one-word = basaloid) - >50% of cells are basaloid Stains: IHC for MSH2, MSH6, MLH1 and PMS2 - loss of expression of mismatch repair proteins suggestive of Muir Torre (not diagnostic) adipophilin, EMA

Answer 454

DAMPED Hair Diabetes Asian Marfans Pregnancy EDS Down syndrome Scarring alopecia Small, firm, skin-colored papule, commonly multiple, may be eruptive Periorbital, trunk, genital

Answer 455

Proliferation eccrine duct structures in dermis Can resemble tadpoles when sectioned at an angle May have horn cysts Stroma often fibrotic or sclerotic Confined to superficial dermis

Answer 456

Soles, palms, scalp (e.g. in sebaceous naevus) sessile vascular plaques surrounded by thin indented moats Can mimic pyogenic granuloma Histo: Cuboidal or basaloid "poroid" cells In lower portion of acanthotic epidermis, extending into dermis Well circumscribed/sharp cut-off May have clear tumour cells Small sweat ducts usually present within tumour Can have small collections of cells in coagulation necrosis - necrosis en masse Stain: CEA - ductal differentiation

Answer 457

solitary dermal or subcutaneous nodule may appear cystic/ have serous draining Histo: Nodular tumour in dermis Mainly one cell type of basaloid cells Sclerotic stroma/hyalinised collagen Can have clear cell change Sweat duct lumina in tumour Excision (benign, but rare reports of extension to LNs)

Answer 458

Papillomatous epidermis, connecting to underlying tumour Cystic space within tumour opens to surface of skin Lined by squamous epithelium in upper portion Lower portion lined by sweat glandular epithelium Apocrine decapitation secretion Papillary projections into cystic spaces Plasma cells around tumour Can be background naevus sebaceous

Answer 459

Brooke Spiegler multiple cylindromatosis Tumour of basaloid cells in dermis, arranged in islands, fit together like a jigsaw puzzle Two types basaloid cells - one is larger with a paler nucleus than the other Sweat duct lumina within islands Hyalinised droplets in island Hyalinised/eosinophilic cylinder around island

Answer 460

Lip, nose Large Basaloid strands Sweat ducts, lumina Horn cysts Deeply infiltrative PNI common

Answer 461

Sharply demarcated nodules of basaloid cells in dermis (or subcut) No connection with epidermis Two types basaloid cells - one is larger with a paler nucleus than the other Basaloid cells arranged in rosettes Sparse small sweat ductal lumina

Answer 462

2 major Or 1 major and 2 minor Or 1 major + genetic confirmation Major - CROP BF Calcification of falx cerebri *Rib abnormalities* (now minor) - bifid, fused, splayed Odontogenic keratocyst (prior to gae 20) Palmar pits (3 or more) BCCs prior to age 20 years or multiple First degree relative with BCNS Minor - FROOMS Frontal bossing (now more cleft/lip palate) Radiological changes - bridging of sella turcica, flame shaped lucencies hands and feet Ovarian/cardiac fibromas + Lymphomesenteric cysts Ocular - strabismus, cataracts, glaucoma, hypertelorism *Meduloblastoma* (now major) Skeletal abnormalities (winged scapula, vertebral fusion, kyphoscoliosis, syndactyly)

Answer 463

AD (50% chance of passing on) De novo in 20-40%

Answer 464

Facial milia Epidermal cysts Multiple naevi Basaloid follicular hamartomas (+BCCs and palmar pits)

Answer 465

Partly depends on gene involved Derm - FSE yearly and after first BCC every 3-6 months Oral - If PTCH1, OPG age 8 or at time of dx and then every 2 years until age 22 (No OKCs with SUFU) Neuro - Neuro exam, if SUFU (or gene unknown) then MRI cerebrum 4monthly until age 3, then 6monthly until age 5 (risk medulloblastoma in PTCH1 1.2-2.4%, 20 times higher with SUFU) Genetics, gen paeds - growth and development Cardiology - cardiac USS at dx, repeat as indicated (3-5% will develop cardiac fibroma, mean age 0-1 month) Gynae - pelvic USS if indicated/symptoms (13-60% ovarian fibroma) Ophthalm - exam at time of dx and repeat as indicated Psychology

Answer 466

PTCH1 (50-70%) or SUFU (4%) Test for PTCH1 Then SUFU Then DNA from at least 2 BCCs can be genetically tested for PTCH1 and SMO

Answer 467

Risk factors in BWH: PNI >0.1mm or named nerve Invasion beyond subcutis Poorly differentiated >2cm diameter T1 = 0 risk factors T2A = 1 RF T2B = 2-3 RF T3 = 4 RF (or bone invasion) Strength of AJCC is determining low risk, or who does not need follow up (it picks up a larger cohort with 'severe disease') BWH has higher predictive value for those at risk of mets (but less sensitivity)

Answer 468

Clark levels 1 - in epidermis (to DEJ) 2 - reaches papillary dermis with slight invasion 3 - fills papillary dermis 4 - invades deep reticular dermis

Answer 469

Recurrence Invades beyond subcut Poorly differentiated PNI Ear or lip Inadequate margins and further surgery problematic Size, >2cm diameter

Answer 470

Hyperkeratosis Sometimes ulceration Parakeratosis Over atypical keratinocytes Sparing epidermis over adnexa (alternating pink and blue in stratum corneum, called flag sign) Atypical keratinocytes (mostly lower portion epidermis) - loss of orderly maturation, hyperchromatism, pleomorphism, mitoses, dysskeratosis Solar elastosis in dermis And perivascular or lichenoid lymphocytes, occasional plasma cells

Answer 471

Parakeratosis (often confluent) Hyperkeratosis Acanthosis Atypical keratinocytes (full thickness) - hyperchromatism, pleomorphism, mitoses, dyskeratosis, loss of orderly maturation Dermis - perivascular or lichenoid lymphocytes or plasma cells

Answer 472

Invasion of dermis by atypical keratinocytes (hyperchromatic, pleomorphic, epithelioid, atypical mitoses) Squamous eddies or keratin pearls Variable perivascular or lichenoid or diffuse lymphocytes or plasma cells Look for PNI

Answer 473

Pink background Arborising vessels Short fine telangiectasias Shiny white blotches and strands Erosions Blue grey dots, blue ovoid nests Leaf like pigment structures Spoke wheel Tan pigmentation or brown granular pigmentation Yellow-white globules Morpheic/infiltrative - background pale sclerotic stroma

Answer 474

Hyperkeratosis, papillomatosis, acanthosis Horn pseudocysts Melanin Sharp demarcation of base epidermis

Answer 475

Hyperkeratosis, papillomatosis, acanthosis, sometimes hypergranulosis Rarely, epidermolytic hyperkeratosis Rarely, acantholytic dyskeratosis Perivascular lymphocytes

Answer 476

Cornoid lamellar = column of parakeratosis, with hypOgranulosis underneath, and dyskeratotic keratinocytes Epidermis in centre lesion can be normal, hypertrophic or atrophic Can get perivascular or lichenoid lymphocytes in dermis (often below cornoid lamellar)

Answer 477

Psoriasiform hyperplasia with clear cells Sharp demarcation from normal epidermis Can get neutrophils in epidermis and microabscesses in stratum corneum Dilated blood vessels in oedematous dermal papillae Perivascular lymphocytes Stain positive PAS

Answer 478

Pale staining Paget's cells throughout epidermis No dyskeratosis Positive for CEA Look for underlying adenocarcinoma in dermis

Answer 479

Keratinising features around follicles/follicular prominence - small white circels, keratin, brown granular pigmentation May have background erythema between follicles with white hollow Multiple focal yellow-brownn keratotic aggregates surrounded by brown ring

Answer 480

Characteristic keratin rim (histo: cornoid lamellar) May have vessels at periphery - looped/hairpin/linear/coiled/glomerular/dotted

Answer 481

Dotted and coiled or glomerular vessels, regularly arranged, string of pearls Pink homogenous background

Answer 482

Grey granular pigmentation Grey dots uniform distribution, peppering

Answer 483

Coiled or glomerular vessels Hyperkeratosis Pigmented - multiple brown dots linear, radiating peripherally Pigmented arches - feather sign

Answer 484

White circles Erosions/ulceration Hairpin/looped or irregular/polymorphous vessels

Answer 485

UV Smoking HPV Arsenic Radiation Immunosuppression Increasing age Skin PT Genoderms/genetic predispositions Longstanding inflammation (chronic nonhealing wounds, DLE, LP, LS, porokeratosis, naevus sebaceous)

Answer 486

Xeroderma pigmentosum Oculocutaneous albinism Gorlins Ferguson Smith Muir Torre epidermodysplasia verruciformis Dystrophic EB

Answer 487

5FU 5% cream BD 2-4 weeks Imiquimod 5% cream twice weekly for 16 weeks Diclofenac 3% gel, Solaraze, BD for 90 days PDT Cryosurgery Curettage, curettage + cautery Chemical peel e.g. TCA Shave excision

Answer 488

If <2cm and not high risk site (periocular, digit, penile): 5-FU 5% cream - 1-2x daily 3-4 weeks Imiquimod 5% cream 3xper week for 4 weeks, may need prolonged up to 12 weeks Cryotherapy 20-30s freeze or 2 cycles of 10-20s Beware prolonged healing, ulceration C+C PDT Esp for poor healing sites, multiple or large lesions Surgical excision 3-5cm margin If high risk site where tissue conservation is important (eyes, nail): Mohs Not first line, but other options: Ablative CO2 Radiotherapy, brachytherapy

Answer 489

often multifocal; can arise in sun-protected areas; also have plamoplantar keratoses; guttate hypopigmentation superimposed on hyperpigmentation

Answer 490

Epithelioma cuniculatum - plantar surface of foot Giant condyloma acuminatum of genitalia, AKA Buschke-Lowenstein tumour Oral florid papillomatosis

Answer 491

Classic Mucosal Giant Subungual Centrifugal marginatum Muir Torre - associated Gryzbowski Ferguson-Smith

Answer 492

Exclude any malignancy (biopsy if needed) Goals: reduce inflammation heal erosions halt progression of scarring alopecia establish realistic expectations relapses and recurrences may occur protect from trauma, sun exposure Gentle soaks to remove crusts - saline, Condy's, Burows, acetic acid Can use LA Treat secondary bacterial infection Potent or ultrapotent TCS OD or BD TCI If refractory: Pred e.g. 4 weeks wea ILCS Acitretin, isotretinoin Dapsone up to 100mg daily Doxy 100mg BD Zinc PDT (beware risk of trauma exacerbating things)

Answer 493

XLD (mostly) Bazex-Dupre-Christol: Follicular atrophoderma -- Usually as circumscribed areas on dorsal hands and feet Hypotrichosis Localised hypohidrosis Milia Epidermoid cysts Multiple BCCs Rombo syndrome: similar features Atrophoderma vermiculatum like appearance on cheeks Hypotrichosis Peripheral (facial, acral) telangiectatic erythema Milia Trichoepitheliomas BCCs Blepharitis

Answer 494

Aluminium acetate 1:40 dilution in water

Answer 495

Potassium permanganate final use concentration of 1 in 10,000 final solution should be light pink

Answer 496

Disorders of cornification in which abnormal differentiation and desquamation of the epidermis result in a defective cutaneous barrier

Answer 497

DRINESS Drugs Renal failure Infection - HIV, leprosy, LGV lymphogranuloma veneream (chlamydia) Neoplasm - lymphomas, MF, KS Nutrition - essential fatty acid def, vit a def, hemochromatosis, marasmus Endocrine - hypothryoid, hypoparathyroid, hypopituitary Eczema Sarcoid Solar damage SLE Sjogrens

Answer 498

CRACKLS Clofazamine Retinoids Allopurinol Cholesterol lowering meds - statins Kava Lithium Spironolactone, frusemide

Answer 499

ADVERBS Vulgaris Erythrokeratoderma variabilis Reticular ichthyosiform erythroderma (ichthyosis with confetti) BIE = epidermolytic ichthyosis Siemens

Answer 500

ARLiCH Lamellar ichthyosis CIE Harlequin

Answer 501

XLD: Conradi Hunerman Happle; CHILD IFAP XLR: X linked ichthyosis

Answer 502

Syndromic: Netherton's Sjogren Larssen Neutral lipid storage disease Trichothiodystrophy Kalmans Refsums Multiple sulfatase deficiency Skin: Ichthyosis vulgaris X linked ichthyosis Lamellar ichthyosis Epidermolytic ichthyosis (Epidermolytic epidermal naevi) Ichthyosis hystrix Superficial epidermolytic ichthyosis (Siemen's) Congenital ichthyosiform erythroderma Harlequin ichthyosis Ichthyosis with confetti Erythrokeratodermas: KID syndrome Erythrokeratoderma variabilis Erythrokeratoderma progressiva XLD: Conradi Hunerman Happle CHILD IFAP

Answer 503

FLG, AD Scaly, not red Sparing of flexures Fine white scale Hyperlinear palms KP, atopic dermatitis Histo: Mild orthokeratotic hyperkeratosis Often diminished granular layer IHC: diminished or absent filaggrin

Answer 504

General: * Hydration: baths, soaks, humidifier. * Lubrication: emollients * Keratolytics: mild abrasives, SA, urea, propylene glycol, LA, retinoids, calcipotriol * Nutrition: iron, protein * Heat intolerance: spray bottles, cooling vests * Anti-infectives: monitor for infection. Low threshold for culture * EHK care: mechanical blistering. Footwear, clothing * Psychosocial: support groups, education, career counselling, sports and recreation. Financial - clothing, cleaning * Genetics: genetic counselling, prenatal diagnosis * Other: alopecia, eyes, ears, physio (contractures), surgical release of pseudoainhum/thorax * Eye: observe for ectropion. Ask about eyelids not closing during sleep * Ear: enquire re intermittent hearing. Ref to ENT for aural toilet * Scalp and hair: scaling and hair loss. Monitor for tinea Growth monitoring: in paeds Topical: * Emollients * Hydration: glycerol, propylene glycol <20%, urea <5% * Lubricating: petrolatum, paraffin. Caution: large areas, can get heat intolerance, maceration, infection * Keratolytics. Urea 10-40% (beware <1yo), propylene glycol >20%, AHA (lactic, glycolic 5-12%), SA (2-5%, 25% - not <2yo) * Topical retinoids - keratinocyte differentiation. * Calcipotriol. <100g/week Targeted topicals - eg cholesterol + lovastatin for CHILD Systemic: * Acitretin 0.5mg/kg/day for scale, hyperkeratosis Isotretinoin, alitretinoin

Answer 505

Steroid sulfatase (STS) XLR Scaly, not red Spares flexures Brown polygonal adherent scale, plate-like Pre-auricular and neck involvement Comma shaped corneal opacities (also in 50% female carriers) Obstructed labour Cryptochordism Does not subside with age - unlike ichthyosis vulgaris Palms, soles, face otherwise spared FISH test to identify gene

Answer 506

TGM1 (thank god it's Monday) AR Scaly, not red HAS flexural involvement Large mosaic plate like scale Heat intolerance Collodion baby PPKD - variable Ectropion Scarring alopecia Nail dystrophy

Answer 507

TGM1 among other genes (10-30% ALOX12B, ALOXE3) Usually AR Erythematous and scaly (fine white) Heat intolerance, hypohidrosis Erythroderma Alopecia (scarring) Ectropion, keratitis PPKD Collodion baby Risk SCCs, BCCs Nails mild Dermatophyte infections

Answer 508

ABCA12, AR Thick yellow brown scale Collodion baby Premature delivery High risk death from sepsis, respiratory failure Ectropion and eclabium Hypoplasia/rudimentary development of nasal and auricular cartilage Sometimes microcephaly Becomes similar to CIE but more severe Erythematous, scaly Painful PPK Dystrophic nails Poor hair growth Hair follicles - concentric accumulation of keratotic material around hair shafts. Highly characteristic

Answer 509

Keratin 1, 10 AD Other name: congenital reticular ichthyosiform erythroderma Erythroderma PPKD Increasing 'normal' spots Increased risk NMSC in the 'normal' skin (early adulthood 20%)

Answer 510

ALDH3A2, AR (aldi has 3 apples in aisle 2) Ichthyosis Spastic tetraplegia, epilepsy, 'scissor' gait Glistening dot atypical retinitis pigmentosa Dental enamel dysplasia Very itchy (from increased leukotriene B4) Ichthyosis favours lower abdomen (esp periumbilical), neck (esp sides and nape), flexures ~70% develop PPK MDM derm, neuro, ophthal, ortho, developmental support

Answer 511

KRT1 and KRT10 AD At least 50% sporadic mutations KT1 has PPK, KT10 does not Blisters, erythema, fragility at birth Dark warty scales Linear spiny ridges in flexures Cobblestone keratoses acrally 'corrugated cardboard' scale Malodour Epidermolytic hyperkeratosis (Large epidermal naevi should be biopsied to look for EHK on path - these patients have mosaic variant of epidermolytic ichthyosis. Therefore they are at risk of having children with 'full blown' epidermolytic ichthyosis if their gonads are involved). As neonate - treat as per EB

Answer 512

K2e AD Mauserung scale Spares palms and soles No odour Rippled hyperkeratotic limbs

Answer 513

KRT1 AD BIE without blisters No odour Porcupine-like scale on extensors Affects flexures PPKD - mutilating, contractures, pseudoainhum, starfish-like keratoses, knuckle pads Nail dystrophy Cobblestone-like or ridged surface over large joints and in skin folds

Answer 514

Ichthyosis, Brittle hair, Intellectual impairment, Decreased fertility, Short stature AR ERCC2, ERCC3 Photosensitivity in 50% Polarised light microscopy- 'tiger tail hair' Regular light microscopy - trichoschisis, can get trichorrhexis nodosa (*if you see tiger tail hair then look for these things and perhaps just mention trichoschisis) low sulfur content hair Other: Neonatal erythroderma, collodion Hypohidrosis Folliculitis Pruritus Nail changes - ridging, thinning

Answer 515

SPINK5 AR Erythematous +/- Collodion Atopy - eczema, food allergies Itch Ichthyosis linearis circumflexa trichorrhexis invaginata (can also get trichorrhexis nodosa, pili torti) Ectropion, keratitis, blepharitis Hypernatraemia FTT Elevated IgE, eosinophilia

Answer 516

GJB2,6 AD Keratitis Ichthyosis Deafness Periorificial plaques, radial furrows PPKD Risk SCCs Porokeratotic eccrine ostial and dermal duct naevus (PEODDN)

Answer 517

AR Ataxia Deafness Developmental delay Myopathy HSM Cataracts Lipid containing vacuoles in leukocytes

Answer 518

X linked recessive Obesity Anosmia Hypogonadism (+ Ichthyosis obvi)

Answer 519

Phytanic acid deficiency Salt and pepper retinitis pigmentosa Cardiac arrhythmia Ataxia, neuropathy (+ Ichthyosis obvi)

Answer 520

FTT Developmental delay Neurological deficits Premature death in child

Answer 521

Ichthyosis Follicular keratoses Alopecia Photophobia (+keratitis)

Answer 522

GJB3, GJB4 AD Fixed and migratory plaques, geographic PPKD

Answer 523

AD GJB4 Mild PPKD Fixed plaques

Answer 524

EBP X linked dominant Short stature Asymmetric cataracts, asymmetric skeletal abnormalities Dysmorphic facies, frontal bossing, macrocephaly Follicular atrophoderma replacing blaschkoid hyperkeratosis Alopecia Chondrodysplasia punctata Erythroderma at birth

Answer 525

Congenital Hemidysplasia With Ichthyosiform Erythroderma [or Nevus] and Limb Defects NSDHL XLD Unilateral ichthyosiform erythroderma Sharply demarcated at midline Spares face Stippled epiphyses Ipsilateral alopecia, limb/visceral hypoplasia Verruciform xanthomas claw like nail dystrophy, onychorrhexis (vertical ridges on nails) Treatment: topical lovastatin or simvastatin plus cholesterol

Answer 526

PORCN XLD Like IP, males can only survive with it in the case of Klinefelter's or postzygotic mutation Skin, eyes, teeth, bones Dermal atrophy variable fat “herniation” telangiectasias pigmentary changes (hypo/hyper) “Raspberry-like” papillomas - perioral and anogenital regions Xray midportion lower legs - osteopathia striata syndactyly, oligodactyly Eyes - often unilateral, include iritic and chorioretinal colobomas, microphthalmia, anophthalmia, and cataracts abnormal teeth (e.g. vertical grooving, hypodontia) Nail dystrophy

Answer 527

Need to NAB PAPA or DIE Non-syndromic (sporadic >AD) (AD gene is BMS1 (be my soul 1)) Neavus - epidermal, sebaceous, large CMN, organoid Adams Oliver syndrome CMTC, malformation limbs, nail dystrophy, syndactyly, brachydactyly, oligodactyly, cardiac defects, pulmonary hypertension, developmental delay Bart syndrome EB + aplasia cutis Papyraceous fetus Antiphospholipid syndrome Placental infarct Amniotic bands Drugs - heparin, marijuana, cocaine, valproate, carbimazole, misoprostol Infections - HSV, VZV, rubella Encephalocoele, heterotopic brain, meningocoele, other embryological malformation

Answer 528

XLD HCCS ("have cornea that can't see") microphthalmia, dermal aplasia, sclerocornea, and sometimes cardiac arrhythmias

Answer 529

Inflammatory Linear Verrucous Epidermal Nevus linear, erythematous, scaly plaque follows the lines of Blaschko characteristically pruritic can get ipsilateral skeletal abnormalities - usually limb reduction possible association with arthritis Histologically, psoriasiform hyperplasia with alternating parakeratosis and orthokeratosis

Answer 530

TB Chancre - no prior TB exposure - from inoculation/injury - brown-red papule - turns into ulcer, undermined edge - painless TB verrucosa cutis - verrucous plaque at site of inoculation, asymptomatic - feet/legs/buttocks +/- Lupus vulgaris - head and neck (and legs) - red-brown plaque - atrophic centre - one scaly/crust edge, one scarred/fibrosed edge - asymptomatic - apple jelly diascopy - some immunity/prev sensitised - may have CNS or abdo TB (~10%) - SCC possible complication

Answer 531

Acute miliary TB - Unwell - Polymorphic papules, pustules, nodules - May have central crust, umbilication - Trunk and limbs - Haematogenous spread - Immunosuppressed (may have HIV) Orofacial TB - Contiguous, autoinoculation - Mouth, anal, genital - Non-healing, usually painful, punched out ulcers - Immunosuppressed - Almost 100% have lung involvement TB Gumma/metastatic abscess - Subcutaneous nodules - Haematogenous spread - Immunosuppressed - Abdo involvement 50% Scrufuloderma - Subcutaneous nodules, undermined ulcers with blue edge, fistulae - Contiguous spread from LNs or bones/joints - Puckered scarring - A/w pulmonary, bone/join, CNS, abdo TB +/- lupus vulgaris

Answer 532

Lichen scrufulosorum - Multiple perifollicular papules grouped - Erythematous, scale crust - Asymptomatic - Trunk and proximal extremities Erythema induratum (of Bazin) - Posterior lower legs young women - Subcutaneous nodules, plaques, ulceration - Tender Papulonecrotic tuberculid - Papules, pustules, nodules - Painless - Erythematous - violaceous - Recurring crops - Varioliform scars

Answer 533

Mycobacterium tuberculosis Mycobacterium bovis BCG (bacille Calmette–Guérin)

Answer 534

- Histology Caseating necrosis Giant cells (Langhans) Epithelioid tuberculoid granulomas - Stains AFB Ziehl Neelson, Fite Auramine-rhodamine - QFG (interferon gamma release assay) - PCR - Culture - Tuberculin skin test - FNA - Ix for TB elsewhere CXR, CT chest, abdomen, MRI CNS, sputum cultures/PCR/AFB smear

Answer 535

Rifampicin - Orange secretions - Hepatitis - SJS/TEN - DRESS - Cutaneous vasculitis - Urticaria Isoniazid - Hepatitis - Peripheral neuropathy - SJS/TEN - DRESS - Lichenoid drug eruption Pyrazinamide - Hepatitis - Arthralgia - Phototoxicity - SJS/TEN - DRESS - Lichenoid drug eruption Ethambutol - Optic neuritis - Peripheral neuropathy - SJS/TEN - DRESS Bedaquiline, pretomanid, delamanid (options for resistance) - QT prolongation

Answer 536

Deposits of amorphous, acellular material in dermis Needle-like spaces/clefts within deposit Histiocytes and multinucleated giant cells surrounding deposits +/- lymphocytes Secondary calcification or even ossification may be seen 20% silver nitrate solution - crystals are black in surrounding yellow material De Galantha stain ethanol-based fixative preserves crystals crystals exhibit negative birefringence when examined by polarized light

Answer 537

General: diet (reduce meats), no ETOH, lose weight Indomethacin (50mg TDS) Colchicine Chronic: allopurinol

Answer 538

diarrhea, thrombocytopenia, hepatitis Skin (HLA-B*58:01): SJS/TEN DRESS Also: Morbilliform Lichenoid Ichthyosis CSSV

Answer 539

AR ECM1 extracellular matrix protein 1 (every child matters) South Africa Deposition of hyaline material in skin, mucosa, larynx, internal organs 1st sign: weak cry or hoarseness - infiltration of laryngeal mucosa Skin signs during first 2 years of life: Vesicles and hemorrhagic crusts Face, mouth, extremities Scarring - ice-pick Second stage: Skin diffusely thickened and waxy; develops yellow colour Papules, plaques, nodules on face (eyelid margin) + axillae + scrotum Verrucous lesions - elbows, knees, hands Generalised scale Alopecia eyebrows and eyelashes Corneal ulcers Diffuse infiltration tongue Frenulum involvement - can't stick out tongue Occlusion of salivary duct, recurrent parotitis Loss of teeth Neuro: seizures, behaviour difficulties, intracerebral hemorrhage, sickle-shaped calcification within the amygdalae Normal life expectancy

Answer 540

Hyperkeratosis Papillomatosis Amorphous eosinophilic deposits, beginning around vessels then diffuse, around adnexal structures Perpendicular to epidermis (vertical) Staining: alcian blue, colloidal iron, PAS deposition of amorphous or laminated material around blood vessels and within the connective tissues

Answer 541

ENT Neuro, antiepileptics Sunprotection Dermabrasion CO2 laser Chemical peels Oral, IL and topical corticosteroids Oral retinoid Penicillamine Oral dimethyl sulfoxide (DMSO)

Answer 542

Hunter XLR Hurler AR Coarse facies intellectual disability Hepatosplenomegaly skeletal abnormalities (dysostosis multiplex) joint stiffness cardiovascular disease corneal clouding Skin-coloured to white papules over the scapulae (pebbling) Extensive dermal melanocytosis Hypertrichosis Histo: Granules in cytoplasm of fibroblasts or histiocytes - stain +ve toluidine blue, alcian blue, colloidal iron, giemsa Mucin in dermis May need fixation in alcohol

Answer 543

Adult, juvenile, pigmented, nodular dome-shaped, translucent papules in sun-exposed skin Histo: nodular fissured masses of amorphous eosinophilic material solar elastosis similar staining to amyloid Grenz zone

Answer 544

(more common first) Papular, plaque, lupus pernio, subcutaneous (Darier-Roussy), scar/tattoo (then list the others) Hypopigmented Ulcerative Maculopapular, mucosal Annular, atrophic, alopecia, angiolupoid Nodular, nail Subcutaneous (Darier-Roussy) Papular, plaque, palmoplantar, psoriasiform Erythrodermic, EN Lupus pernio, lichenoid Verrucous Ichthyosis Scar, tattoo

Answer 545

non-caseating epithelioid granulomas naked Multinucleated histocytes/giant cells usually Langhans type, nuclei arranged in peripheral arc or circular fashion Asteroid or schaumann bodies in giant cells

Answer 546

IFN TNF Immune checkpoint inhibitors Targeted kinase inhibitors IL1 Anti-retrovirals

Answer 547

LEAF Fever, arthritis, hilar lymphadenopathy and EN (+/- uveits) Supportive measures, systemic corticosteroids Spontaneous resolution over 1-2 years

Answer 548

Most common nose, cheeks, ears (then lips, forehead, fingers) Lung/resp tract sarcoid Also a/w uveitis, bony cysts of terminal phalanges, dactylitis, nail dystrophy

Answer 549

Parotid gland enlargement Uveitis Fever Cranial nerve palsies (usually facial nerve)

Answer 550

Lacrimal/salivary gland enlargement Xerostomia Xerophthalmia

Answer 551

Bell's palsy Granulomatous cheilitis Fissured tongue

Answer 552

Childhood onset CARD15/NOD2 mutation, AD Uveitis Arthritis Constitutional symptoms

Answer 553

Lungs - 90% - CXR, HRCT, PFTs Hilar and/or paratracheal lymphadenopathy - 40% - CXR, CT Eyes - 25% - uveitis, conjunctivitis, sicca - annual ophthalm Cardiac - 5-25% - palpitations, arrhythmias, CHF, sudden death - ECG, Holter, echo Liver/spleen - 10-20% - LFTs, plts, CT abdo CNS + PNS - 10-20% - neuropathies - neurologist, NCS, MRI Upper resp tract - 5-10% - sinusitis, nasal congestion Bones - 5-10% - lytic bone lesions Joints/muscles - 5-10% - arthritis Bone marrow - 50% - lymphopaenia -FBE, SPEP (lymphoma association) Kidneys - 10-40% - nephrolithiasis, renal impairment - UECs, 24hr urine calcium, serum calcium Endocrine - 5-10% - pituitary, thyroid, hypercalcaemia - TFTs

Answer 554

General measures ○ MDT ○ Exclude drug induced ○ Regular monitoring Cutaneous involvement ○ EN: NSAIDs, PNL, rest ○ Topicals , ILCS Other systemics (other than PNL) ○ Antibiotics - mino, doxy ○ HCQ ○ MTX, MMF ○ Leflunomide ○ Thalidomide ○ Apremilast ○ Pentoxifylline ○ Allopurinol ○ Isotretinoin ○ JAK inhibitors ○ TNF inhibitors Other/adjuncts: Phototherapy, laser, PDT, surgery

Answer 555

Biopsies FBE, UEC, LFTs, calcium ACE (2-3x ULN, elevated in 60%, false +ve 10%) 24 hr urinary calcium, urinalysis TSH Vit D QFG SPEP, UPEP CXR, lung function test CT chest Ophthalm review for eyes ECG, Holter, echo NCS/MRI Xrays as needed for joints/bones

Answer 556

Chronic course in 10-30% Lupus pernio - associated with chronic disease Acute presentation e.g. Lofgrens - typically resolves in 2-5 years

Answer 557

EF PLC SPRAINED Eczema Fungal Psoriasis Lichen planus Climacteric (keratoderma climactericum) SLE/DM PRP Reiter's (keratoderma blennorrhagicum) Acanthosis nigricans Infections - syphilis, scabies Neoplastic - MF/sezary, bazex Endocrine - hypothyroid Drugs (imatinib, trametinib, vemurafenib)

Answer 558

keratoderma blennorrhagicum AUC Arthritis Urethritis Conjunctivitis Circinate balanitis or vulvitis Psoriasiform rash elsewhere

Answer 559

CLAW DRIPS Callosities Lichen planus/LE Arsenic Warts Drugs Reiters Infection - tinea Porokeratosis, psoriasis Spiny keratoderma (malignancy, CKD and PCKD)

Answer 560

Gorlin's Darier's Punctate PPK Pitted keratolysis Punctate porokeratoses

Answer 561

FLIC B T Flu vaccine Lithium Iodide Calcium channel blockers BRAF inhibitor TNF inhibitors

Answer 562

1. Is there transgrediens? If yes, mal de meleda or Greithers Then check mouth and eyes (periorbital and perioral erythema with Mal de Meleda) If no transgrediens, then biopsy looking for epidermolytic hyperkeratosis If EHK, then Unna Thost Vorner If no EHK, then BoNK Bothnia Nagashima Kimonis

Answer 563

Isolated: Striate/focal Syndromic: COPS-T Carvajal Oculocutaneous tyrosinaemia (Richner Hanhart) Pachyonychia congenita SAM syndrome Tylosis/Howel-Evans

Answer 564

SHuKs Sex reversal + SCC Huriez KLICK

Answer 565

Hearing loss? No - Loricrin Yes (HUMB) Vohwinkle Mitochondrial Bart Pumphrey

Answer 566

CHOPS Cardiac (Naxos) Hidrotic ectodermal dysplasia (Clouston) Olmsted Papillon-Lefevre Shopf schulz passarge

Answer 567

Syndromic: Cole syndrome Isolated: BAFS Buschke-Fischer-Brauer Acrokeratelastoidosis Focal acral hyperkeratosis Spiny keratoderma

Answer 568

AD Diffuse PPK Transgrediens Glove and stocking Pseudoainhum Mutilating May improve post puberty

Answer 569

AR SLURP1 Diffuse PPK Transgrediens Glove and stocking Pseudoainhum Malodour Periorbital, perioral erythema

Answer 570

AD KRT1,9 No transgrediens Epidermolytic hyperkeratosis Waxy red border Onset first year of live

Answer 571

AD presents at birth PPKD sclerodactyly/atrophy loss of dermatoglyphics transgrediens nail dystrophy SCC (skin + oesophagus >100x normal) Hyperhidrosis

Answer 572

AR POMP Keratosis linearis with ichthyosis congenita and sclerosing keratoderma Linear hyperkeratosis Ichthyosis at birth PPK with pseudoainhum and sclerosing flexion deformity risk SCC

Answer 573

AD TRPV3 Ectodermal PPK Transgrediens Mutilating PPK with prominent periorificial keratotic plaques + perianal SCC and melanoma risk Alopecia, nail dystrophy

Answer 574

AD GJB2 connexin 26 Honeycomb PPK Starfish keratoses Pseudoainhum Deafness (Alopecia, nail, myopathy)

Answer 575

AR RSP01 PPK, sclerosis SCC risk

Answer 576

AD GJB2 connexin 26 Honeycomb PPK Knuckle pads Deafness Leukonychia

Answer 577

MTTS1 Honeycomb PPK Deafness

Answer 578

AR Plakoglobin (JUP) Diffuse PPK Woolly hair Right sided cardiomyopathy Arrhythmias

Answer 579

AR WNT10A Other name: odonto-onycho-dermal dysplasia Apocrine hidrocystomas Hypodontia Hypotrichosis Nail changes/dystrophy SCC, BBC, eccrine syringofibroademona, eccrine poroma

Answer 580

AR CTSC - cathepsin C Transgrediens PPK Glove and stocking Periodontitis (loss of teeth) Pyogenic infection Hyperhidrosis, malodour Psoriasiform plaques

Answer 581

AD GJB6, connexin 30 Cobblestone PPK Hyperkeratosis elbows, knees Alopecia or wiry brittle hair Nail dystrophy Normal teeth and sweating Deafness

Answer 582

AD DSG1, KRT1, DSP (desmoplakin) Onset in adolescence

Answer 583

AR Desmoplakin Woolly hair cardiac arrhythmias Left sided cardiomyopathy Striate PPK

Answer 584

AR TAT Painful callosities soles Weight bearing non transgrediens PPK Photophobia, keratitis, corneal ulcers, blindness Developmental delay Low phenylalamine/tyrosine diet

Answer 585

AD KRT 6A, 6B, 6C, 16, 17 Triad - PPKD (focaal) + toenail dystrophy (wedge subungual hyperkeratosis and omega shape) + plantar pain, difficulty walking Also laryngeal involvement 17 - steatocystomas, vellus hair cysts, epidermoid cysts 6A, 16 - oral involvement Oral leukokeratosis Angular chelitis Natal teeth Follicular keratosis of knees, elbows

Answer 586

AD RHBDF2 Mostly soles, pressure points Increased risk oesophageal cancer Oral leukoplakia

Answer 587

Desmoglein 1 Severe dermatitis, allergies, metabolic wasting

Answer 588

AD ENPP1 Punctate PPK Guttate hypopigmentation Ectopic calcification

Answer 589

Buschke Fischer Brauer AD AAGAB Later onset 10-30years Sites of pressure Acrokeratoelsatoidoisis AD Elastorrhexis on histo Cornified papules, umbilicated border Focal acral hyperkeratosis AD African Marginal keratoderma, palm lines Spiny keratoderma AD Cornoid lamellar on histo Multiple tiny spines

Answer 590

XLR DKC1 Mutations affect telomerase Lacy reticulated pigmentation neck, chest, upper arms Premature aging PPK + hyperhidrosis Nail dystrophy (pterygium, ridging, splitting) Leukoplakia (oral, genital) Malformed, missing teeth Epiphora Bone marrow failure Malignancies incl SCC

Answer 591

Inhibits inosine monophosphate dehydrogenase (IMPDH) Targets lymphocytes - T and B cells

Answer 592

Allergy Pregnancy - category D (contraception 1m before, 6 weeks after) Lactation Renal, hepatic, cardiopulmonary disease Drug interactions - AZA, MTX (increased bone marrow suppression) - Cholestyramine, abx (decreases level MMF) - PPI/antacids/iron (decreases level - give MMF 1 hour before) - acyclovir, probenecid (increases level MMF)

Answer 593

MIGHT UC (grabbing a microphone in the hope that might you see) Malignancy Infection GI - dose dependent, most common 20%, nausea, vomiting, anorexia, diarrhoea, abdominal cramps Haem - cytopaenias Teratogenic Urogenital - urgency, frequency, dysuria CNS - headache, tinnitus, weakness, fatigue, insomnia

Answer 594

Inhibition of dihydropteroate synthetase Inhibits neutrophil and eosinophil myeloperoxidase Anti neutrophilic DH LABD Bullous LE EED IgA pemphigus SPD Neutrophilic derms, immunobullous

Answer 595

Hypersensitivity/ Allergy to sulfonamides G6PD Significant renal/liver/cardiopulmonary disease Peripheral neuropathy Pregnancy, lactation

Answer 596

Drug eruptions: morbilliform, DRESS, FDE, SJS, photosensitive, cutaneous lupus, pseudoporphyria Headache Anaemia, haemolytic (dose related and G6PD related) Neuro: visual changes, motor>peripheral neuropathy, psychosis GI, hepatitis, jaundice, nausea, vomiting, anorexia MetHb - dose related Agranulocytosis (idiosyncratic, 3-12 weeks, fever, pharyngitis) Nephrotic syndrome Dapsone hypersensitivity - fever, cutaneous eruption, hepatitis; 3-12 weeks; HLA B13:01

Answer 597

Baseline FBC, EUC, LFT, BHCG, G6PD Follow up 2 weekly 1month then monthly 2 months then 3 monthly Ask about cyanosis, weakness, lethargy, SOB, headache FBE, reticulocytes, UEC, LFTs Methaemoglobin levels as required

Answer 598

Methaemoglobin is a dysfunctional form of haemoglobin Expected, dose related But alarm if cardio/resp symptoms Methylene blue 1-2mg/kg IV, every half hour >20% or >10% and symptomatic Methylene blue – CI G6PD Ascorbic acid Plasma exchange

Answer 599

50mg daily; increase to 100-200mg Tablets: 25mg, 100mg

Answer 600

Dose independent allergic reaction Occurs during first 4 weeks of treatment Systemic symptoms: fever, chills n/v/d arthralgias, myalgias liver, renal dysfunction leukocytosis Cutaneous symptoms: EN Sweets LCV AGEP Non-specific urticaria, pustules, purpura, maculopapular Improves within 5 days stopping Rechallenge contraindicated

Answer 601

CALM - infancy Intertrigenous freckling - infancy and childhood Lisch nodules - early childhood Optic glioma - usually <6 years Neurofibromas - adolescence to adulthood Long bone dysplasia - infancy (congenital)

Answer 602

Subepidermal cell-poor blisters Characteristic festooning of dermal papillae Dermal fibrosis in sclerotic lesions PAS-positive deposits around blood vessels (caterpillar bodies) DIF - often IgG, complement, fibrinogen at DEJ and around blood vessel

Answer 603

AD PPOX (protoporphyringoen oxidase)

Answer 604

KRT5, 14 AD EB simplex localised (palms and soles) EB simplex severe (grouped herpetiform blisters, figurate array, confluent PPK) KRT5 AD EBS with mottling PLEC1 (plectin) AR EBS with muscular dystrophy - nail dystrophy KLHL24 AD EBS with cardiomyopathy - nail dystrophy, marked mucocutaneous fragility at birth, atrophic scarring, alopecia Pyloric atresia

Answer 605

AR laminin 332 or type XVII collagen (COL17A1) LAMA3, LAMB3, LAMC2 severe pyloric atresia - α6β4 integrin severe mucocutaneous disease often fatal in infancy FTT, sepsis nail, hair, tooth abnormalities Excessive granulation tissue, usually symmetric, periorificial areas, skin folds, upper back, nape of the neck, and periungual areas

Answer 606

Can be recessive or dominant COL7A1 (collagen 7) Heals with scarring, contractures, strictures, milia Significant extracutaneous involvement Chronic wounds - chronic pain, inflammation, nutrient loss Variant: Extremely pruritic papules in linear arrangement on lower extremities - DEB pruriginosa

Answer 607

Oral ulcers, pain, poor hygiene, dental carries, microstomia, difficulty eating, dental enamel hypoplasia esp with JEB More with JEB - tracheolaryngeal blistering - potentially fatal airway obstruction GIT oesophageal strictures, poor oral intake, malabsorption, FTT, anaemia, osteoporosis, renal failure due to dehydration, constipation, anal fissures, strictures PEG Ocular Symblepharon, ectropion, corneal abrasion, blindness, blistering, neovascularization Dilated cardiomyopathy Urethral stricture or ureterovesical strictures, ureteric reflux and hydronephrosis Chronic renal failure Vaginal stenosis QoL, mental health, relationships, intimacy, lifelong pain Risk sepsis SCCs Pseudosyndactyly (“mitten” deformities)

Answer 608

70x increased risk SCC Cumulative risk: 7.5% by 20 years, 80% by 45 years Risk of death from SCC: 57% by 35 years, 87% by 45 years UV exposure not implicated Bony prominences, sites of chronic blisters, wounds, scarring Multiple primaries Well differentiated but aggressive behaviour Metastasise despite WLE and clear margins Median survival 2.5 years Median age 30

Answer 609

AR FERMT1 Skin fragility Photosensitivity Poikiloderma Nail and dental abnormalities Oesophageal and urethral strictures Increased risk mucocutaneous malignancy

Answer 610

AR TGM5 Early life Desquamation and flaccid blisters Leaving superficial erythematous erosions Localised palms, soles, dorsums hands and feet Plane of cleavage superficial epidermis - stratum granulosum

Answer 611

MDT - genetics, EB nurse, neonatologists DEBRA NICU admission - Gentle handling - Nurse clothed - Avoid incubator - Avoid adhesive tapes, name bands, clothing seams - Pierce blisters - Gentle dressings - Vinegar soaks, bleach bath - Prontosan - Swab (sens) and abx - Encourage breast feeding, monitor nutrition - Analgesia

Answer 612

BE DR PACK: Blooms EKV - Erythrokeratoderma variabilis DKC - Dyskeratosis congenita Rothmund–Thomson syndrome Poikiloderma with neutrophils Ageing and acquired Cockayne syndrome Kindler GVHD MF DM

Answer 613

Hyperhidrosis - aluminium chloride hexahydrate or botulinum toxin A Non-adhesive or low tack dressings e.g. soft silicone dressings Padding, protection

Answer 614

(1) quiescent/dormant – macular or slightly infiltrated, red, warm lesions mimicking a PWS (2) expansion – warm masses with throbbing and thrills over dilated draining veins (3) destruction – necrosis, haemorrhage, ulceration and (occasionally) lytic bone lesions (plus findings of stage 2) (4) cardiac decompensation (plus findings of stage 2 ± 3)

Answer 615

All AD CMAVM1 RASA1 More CNS/intracranial Classic fast flow/quick refill round pink-red macules with white halo CMAVM2 EPHB4 Mimics HHT Epistaxis Telangiectasias lips, perioral, upper trunk

Answer 616

Indolent - MF - MF variants (folliculotropic, granulomatous slack skin, pagetoid reticulosis) - Primary cutaneous CD30 +ve lymphoproliferative disorders (primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis) - Subcutaneous panniculitis like T cell lymphoma Aggressive - Sezary syndrome - Adult T cell leukaemia/lymphoma - Extranodal NK T cell lymphoma nasal type - Primary cutaneous gamma/delta T cell lymphoma - Primary cutaneous peripheral T cell lymphoma - Primary cutaneous CD8 +ve aggressive epidermotropic cytotoxic T cell lymphoma

Answer 617

Erythroderma Generalised lymphadenopathy presence of neoplastic T cells (Sézary cells) in the skin, lymph nodes, and peripheral blood demonstration of a T-cell clone in the peripheral blood (preferably the same T-cell clone as in the skin) flow cytometry of peripheral blood shows at least 1000 Sézary cells per mm3 CD4:CD8 ratio >10 aberrant expression of pan-T-cell antigens

Answer 618

Anticoagulation e.g. heparin (to prevent clotting in the tubing system) 2 consecutive days each month, assess response after 3 months Takes 2-4 hours Blood is drawn, white cells are separated White cells are treated with PUVA (methoxypsoralen + UVA) Then re-infused back into patient Indications: MF, Sezary, GVHD, SSc

Answer 619

Low BP, dizziness Fever (2-12 hrs) Red, itch (6-8 hrs) Photosensitivity/light sensitivity Anaemia, thrombocytopaenia Take antihypertensive post if needed Fast if hypertriglyceridaemia

Answer 620

Erythroderma marked exfoliation Oedema Lichenification intensely pruritic Lymphadenopathy Alopecia Onychodystrophy palmoplantar hyperkeratosis

Answer 621

Stage 1A <10% BSA Stage 1B >10% Stage 2A clinical lymphadenopathy Stage 2B tumour Stage 3 erythroderma Stage 4 visceral or LN involvement

Answer 622

SKIN BIOPSY FBE, film UEC, LFTs, CMP B2M, LDH Flow cytometry TCR - tissue and blood CD4:CD8 ratio Degree of CD30 expression HTLV1, CMV Hep B, C, HIV SPEP Thyroid (pre-interferon) PET CT

Answer 623

General - bleach baths, analgesia, emollients, gentle clothing, soap free wash, menthol cream, hospital for flares MDT - Pain, pal care, psychiatry, psychology TCS Top chemo - 5-FU, imiquimood 5%, nitrogen mustard 0.1-0.2% nbUVB, PUVA localised radiotherapy TSEB Acitretin (in combo with PUVA) Interferon alpha MTX HDACi (histone deacetylase) - oral vorinostat, IV romidepsin Brentuximab Chemo - doxorubicin, cladribine Alemtuzumab Allogenic HSCT ECP

Answer 624

Age >60 Male Plaques Folliculotropic disease Lymph nodes palpable Nodal, blood or visceral involvement Large cell transformation Thickness of tumour infiltrate Elevated LDH or B2M Large circulating Sezary cells High Sezary cell count Loss of T cell subset markers such as CD5, CD7

Answer 625

Epidermis - normal, atrophic, hyperplastic, ulcerated Epidermotropism - halo nuclei/vacuolated halos Pautrier microabscesses Lichenoid Papillary dermal fibrosis - ropey collagen Atypical cells - cerebriform, hyperchromatic nuclei CD4:CD8 ratio 3:1 Loss of mature T cell marker - CD2,3,5,7

Answer 626

Patch, plaque, tumour Eczematous, psoriasiform Poikiloderma Buttocks, trunk, limbs (bathing suit) Becomes more infiltrated reddish-brown, scaling plaques, may have an annular, polycyclic or typical horseshoe-shaped configuration Nodules, tumours can ulcerate Morphological variants - hypo/hyperpigmented; bullous; folliculotropic

Answer 627

angiotensin-converting enzyme (ACE) inhibitors, β-blockers, metronidazole, and isotretinoin, as well as arsenic, bismuth, gold, barbiturates, clonidine, hydrochlorothiazide, imatinib, omeprazole, terbinafine, TNF-α inhibitors, NSAIDs, and multiple vaccines Think of a bed of roses, as in the secret garden, it is ISOLATED (ISOTRETINOIN) away from the METROPOLITAN (METRONIDAZOLE) area, so no need for the BETA BLOCKERS, really it is ACE. There is a GOLDen key and locket, and a BARBED (barbiturate) wire fence. You can meditate like 'OM' and its good for your soul (OMEPRAZOLE). You can go away there to HIDE (HYDROCHLOROTHIAZIDE). And maybe even have a TNF. Avoid the bee hive/terbine (TERBINAFINE).

Answer 628

Parakeratosis (my be focal/mounded) Mild spongiosis Exocytosis lymphocytes into epidermis Can get intraepidermal aggregate of Langerhans cells in vase or flask shape Perivascular lymphocytic infiltrate Red blood cell extravasation EAC is good ddx

Answer 629

Multiple histopath subtypes Type A - Wedge shaped infiltrate of CD30+ tumour cells + mixed inflammatory cells Reed Sternberg cells (Hodgkin lymphoma - like) Type B - Resembles MF, epidermotropism of atypical lymphoid cells with cerebriform nuclei Type C - Cohesive sheets of CD30+ large atypical lymphoid cells Type D - Epidermotropic infiltrate of CD30+ and CD8+ atypical lymphoid cells Type E - Angiodestructive infiltrate of CD30/CD8 positive cells around central ulcer

Answer 630

Watch and wait TCS ILCS nbUVB MTX Radiotherapy Total skin electron therapy Brentuximab Lifelong follow up - May get a second malignancy eg MF, C-ALCL, Hodgkin lymphoma, CLL, MDS, multiple myeloma, B cell lymphoma

Answer 631

Typically solitary rapidly growing nodule on trunk, face, extremities Can progress to nodal, then visceral disease (10-15%) Histo features in support of C-ALCL (which can be hard to distinguish from large cell transformation in MF): - Perforin + - Abnormal T cell immunophenotype - Galectin 3 expression - ALK-1 + - DUSP22 rearrangement - NPM1-TYK2 gene fusion

Answer 632

Imatinib MTOR - sirolimus MEK inhibitor e.g. trametinib Vascular endothelial growth factor receptors (VEGFRs)

Answer 633

Furfur Globosa Restricta Obtus Sloofiae

Answer 634

Clusters of thick walled round budding yeast like cells (Short) angular hyphal forms (spaghetti and meatballs) Note: with pityrosporum folliculitis, there are yeast forms only (no hyphal forms)

Answer 635

Econazole 1% solution (Pevaryl) - Apply to wet skin, allow to dry, leave on overnight, repeat for 3 consecutive nights Ketoconazole 2% shampoo Selenium sulfide shampoo 2.5% Topical ketoconazole cream 2% (or terbinafine or clotrimazole cream) If extensive/refractory - oral itraconazole or fluconazole

Answer 636

Hortaea werneckii KOH exam - septate pigmented hyphae Treatment: Whitfield's ointment 6% benzoic acid 3% sal acid Compared with acral naevi - generally larger, lighter in colour, and lack linear striations

Answer 637

Black piedra - Piedraia hortae Scalp and face Hard White piedra - Trichosporon spp. Face and pubic/axillary Soft

Answer 638

T mentagrophytes (inflammatory tinea pedis, barbae, cruris, small mammals) Microsporum canis T verrucosum (can mimic bacterial furunculosis, exposure to cattle)

Answer 639

T rubrum T interdigitale E floccosum T tonsurans (in children) Fusarium spp Candida

Answer 640

T rubrum T tonsurans Epidermophyton flocossum T concentricum (causes tinea imbricata) T interdigitale

Answer 641

follicular papulopustules or granulomatous nodules Can be T rubrum

Answer 642

Epidermophyton floccosum, T. rubrum, and T. mentagrophytes

Answer 643

typically zoophilic dermatophytes - T. mentagrophytes and T. verrucosum abscesses, sinus tracts, bacterial superinfection, and kerion-like boggy plaques

Answer 644

Adjunctive treatment for household contacts and patient: - antifungal shampoo, such as 2% ketoconazole or 2.5% selenium sulfide, until free of disease - combs, brushes, headwear disinfected or preferably discarded Treatment: - Griseo 20mg/kg in divided doses, up to 500mg daily - Terbinafine 250mg if >40kg, 125mg if 20-40, 62.5mg if <20kg - Pred 1mg/kg for 3-5 days - Consider abx

Answer 645

Endothrix Watch TV inside Tonsurans (tonsillitis, therefore having night in) Violaceum (violent movie) Lights off - does NOT fluoresce with woods lamp So - Soudanese (International Film Festival) Go get - Gourvilli (gourmet food) Your- Yaoundei (sitting in ya undies) Red - Rubrum Ectothrix Cats and Dogs Fight and Growl outside Canis Audouini - they disappear because they are microscopic Distortum - think Houdini like above Ferrugineum - they are furry Gypseum - also magical, or grey (yellow green fluorescence in FACD) Verrucosum Mentagrophytes

Answer 646

T. schoenleinii bluish-white fluorescence by Wood's light examination Clinically thick, yellow crusts composed of hyphae and skin debris (“scutula”)

Answer 647

Moccasin Interdigital Inflammatory Ulcerative

Answer 648

budding yeast and pseudohyphae

Answer 649

1. Acute pseudomembranous 2. Acute atrophic 3. Chronic atrophic 4. Median rhomboid glossitis 5. Angular cheilitis 6. Linear gingival erythema 7. Hyperplastic

Answer 650

“verrucous dermatosis” Leg > arm Histo: Pseudoepitheliomatous hyperplasia, intraepidermal abscesses, suppurative and granulomatous inflammation within the dermis. Pathognomonic round, pigmented “Medlar bodies” or sclerotic bodies, resembling “copper pennies”, in the dermis Treatment: Surgery, heat therapy, systemic antifungals (long course) incl itraconazole, terbinafine (>6 months)

Answer 651

Due to implantation granulomatous infection characteristic draining sinuses containing grains (sclerotia, sulfur granules) local oedema fungi and bacteria (actinomycosis, nocardia) Foot > hand, trunk, scalp Treatment: Surgery + systemics (including antibiotics if actinomycotic; antifungals if eumycotic - mostly itraconazole) - continued for months to years

Answer 652

Subcutaneous mycosis Schenckii Mexicana Braziliensis Globose Histo: suppurative and granulomatous inflammation in the dermis and subcutis budding yeast and cigar-shaped organisms can often be visualized with PAS Treatment: Potassium iodide Itraconazole

Answer 653

Histo: characteristic chains of thick-walled, yeast-like cells (“brass knuckles”) with intercellular bridges dermal granulomatous infiltrate Treatment: Surgical excision Cryo Not typically responsive to antifungals - can try itraconazole

Answer 654

histoplasmosis, blastomycosis, coccidioidomycosis, and paracoccidioidomycosis (molds in nature and yeasts in living tissue)

Answer 655

Histoplasmosis Granuloma inguinale/donovanosis Rhinoscleroma Leishmaniasis Penicilliosis

Answer 656

Histoplasma capsulatum Soil etc Inhalation or direct Can get pulmonary, disseminated or primary cutaneous Oral ulcers Nodules and vegetative plaques If immunocompromised - multiple erythematous papules or nodules with scale or crust (molluscum like) Histo: Intracellular yeast forms surrounded by rim of clearing Histiocytes and giant cells are host cells Stains - PAS or methenamine silver Treatment: amphotericin B IV - first line itraconazole

Answer 657

Coccidioides immitis and C. posadasii Severe disseminated if immunocompromised (mutations in IFNGR1 or STAT1) Can resemble molluscum in HIV patient flu-like syndrome Favours face EM + EN hypersensitivity reactions Ulcers OM and meningitis Histo: Characteristic endospore containing spherules PAS and methenamine silver suppurative granulomatous reaction with histiocytes, lymphocytes, and giant cells Treatment: Amphotericin B intravenous dose for severe disease is 1.0–1.5 mg/kg per day prolonged therapy with itraconazole (400 mg/day) (or fluconazole if meningitis) Other: Voriconazole and posaconazole

Answer 658

Paracoccidioides brasiliensis Inhalation/pulmonary Mucocutaneous moriform stomatitis Histo: Yeast forms are found within and outside of giant cells Mariner's wheel - large, thick-walled organisms with multiple narrow-based buds

Answer 659

neutropenic patients - disseminated candidiasis, aspergillosis, mucormycosis HIV - disseminated histoplasmosis, coccidioidomycosis, cryptococcosis and infection due to Talaromyces (Penicillium) marneffei

Answer 660

Neutropaenia susceptible, or diabetes Necrotic lesions - angioinvasive Often face 90 degree branching non septate Voriconazole not effective

Answer 661

from avian esp pigeon droppings Ulceration Cellulitis Molluscum contagiosum like in HIV CNS, bone Histo: gelatinous with numerous organisms and little inflammation OR granulomatous with fewer organisms, more inflammation, and little necrosis PAS highlights the central yeast forms Mucicarmine or Alcian blue is used to visualize the characteristic capsule

Answer 662

Pigmented brown to black hyphae

Answer 663

Disseminated superficial actinic porokeratosis Porokeratosis of Mibelli Linear porokeratosis Porokeratosis ptychotropica Punctate follicular porokeratosis Porokeratosis palmaris et plantaris disseminata (PPPD) = variation of punctate porokeratosis Porokeratotic eccrine ostial and dermal duct naevus

Answer 664

Unmyelinated type C Thinly myelinated type A delta

Answer 665

Increased miu opioid And decreased kappa opioid Contributes to itch

Answer 666

TCS Top CNI (TRPV1 agonism) Anaesthetics (pramoxine, lidocaine, prilocaine) - uraemic, neuropathic - Pramoxine 1% lotion - Lidocaine 5% patch, 2.5-5% cream Combined ketamine-amitriptyline-lidocaine (10-5-5%) (risk toxic encephalopathy if applied >50% BSA) Capsaicin (TRPV1 agonism) - 0.025%-1% cream (8% if patch) - needs to be applied three to six times daily for maximal effect Coolants - menthol (1%), camphor (0.5%) Cannabinoids PDE4 crisabarole 2% Doxepin 5% (risk drowsiness if >10% BSA) Top gabapentin 6% Botox injection (2-10IU)

Answer 667

Antihistamines Immunosuppressants - MTX, CsA, MMF, AZA, pred as rescue Dupilumab JAKi Thalidomide (esp PN, uraemic pruritus) Antidepressants - TCAs e.g. amitriptyline, doxepin; SSRIs e.g. sertraline, mirtazapine Anticonvulsants - gabapentin, pregabalin Naltrexone (miu opioid antagonist) Bile acid resins and rifampicin Phototherapy Neurokinin 1 inhibitors - aprepitant (CYP3A4 inducer, expense) Don't forget - acupuncture Transcutaneous electric nerve stimulation CBT

Answer 668

Gabapentin Start 300mg nocte Increase every week Split dose over 2 to 3 per day Up to cumulative 3600mg/d, depending on tolerance (sedating) Pregabalin Start 75mg nocte Increase to BD after 1 week Max 300mg/d Risks: sedation, fatigue, ataxia Caution with elderly Renal adjust (renally excreted)

Answer 669

JAK inhibitors (all) IL-13 - lebrikizumab, tralokinumab IL-31 - nemolizumab

Answer 670

Capsaicin Topical anaesthetics Topical ketmine-amitriptyline-lidocaine Gabapentinoids TCAs Opioid modulators

Answer 671

Topical anaesthetics Gabapentinoids Phototherapy Opioid modulators

Answer 672

Cholestyramine Rifampicin Phototherapy Opioid modulators Sertraline

Answer 673

Capsaicin Phototherapy Beta blockers Opioid modulators

Answer 674

calcium channel blockers HCT checkpoint inhibitors eGFR opioids antimalarials cocaine, methamphetamines

Answer 675

Lobular capillary haemangioma Well circumscribed Exophytic, pedunculated Proliferation of small capillaries, often arranged in a lobular pattern Fibromyxoid matrix/stroma May be bounded by collarettes of hyperplastic epithelium

Answer 676

Scalp dysaesthesia C2-7 Brachioradial pruritus C3-7 Notalgia paraesthetica T2-6 Anogenital pruritus L4-S2 Can become generalised - central neural sensitisation Cheiralgia paresthetica - sensory mononeuropathy of the superficial branch of the radial nerve - dorsoradial aspect of the hand and proximal thumb - Trauma or pressure - watch or cuffs Meralgia paresthetica - Anterolateral thigh lateral femoral cutaneous nerve (pressure as passes under inguinal ligament)

Answer 677

Rare intraepithelial adenocarcinoma - primary (apocrine origin) or secondary to an underlying malignancy Secondary EITHER: underlying visceral malignancy in 10–20% - anorectal, urothelial, cervical, prostatic, ovarian or endometrial OR: cutaneous extension of an underlying adnexal adenocarcinoma

Answer 678

Histology: Vacuolated pagetoid cells in the epidermis, clear or eosinophilic cytoplasm, large pleomorphic nuclei and occasional mitoses look for an underlying adnexal tumour Stains - CEA, CK7 CK20 positive, GCFDP-15 negative favours underlying visceral malignancy CK20 negative, GCFDP-15 positive favours either primary EMPG or secondary to underlying adnexal adenocarcinoma IHC - excl melanoma (SOX10, S100), SCC in situ (CK5/6, p40, p63) Investigations for underlying malignancy: FSE, LNs (USS, FNA) Colonoscopy, cystoscopy In women - complete pelvic and breast examination, imaging (USS, mammogram), cervical screening In men - PSA and prostate exam CEA (colorectal cancer)

Answer 679

WLE or Mohs SLNBx if invades reticular dermis or LV invasion on histo Imiquimod Radiotherapy PDT CO2 laser ablation 5-FU Combo Treat any underlying malignancy Trastuzumab for extramammary Paget disease with HER-2 overexpression

Answer 680

5-year overall survival rate of 75–95% High recurrence rate 30-60% Poor prognostic risk factors: Perianal disease in males aged over 75 years Nodules in the tumour Lymph node involvement either clinically or on histology Paget cells infiltrating the reticular dermis or deeper Long term follow up monitor for local disease recurrence, development of internal malignancy, regional lymphadenopathy, or distant metastasis

Answer 681

2023 EULAR/ACR 3 clinical points and 3 lab points Clinical: 1. Venous thromboembolism - DVT/PE (3 points if no risk factors, 1 point if risk factors) 2. Arterial thrombosis - MI/stroke (4 points if no risk factors, 2 points if risk factors) 3. Microvascular involvement - includes livedo racemosa, livedoid vasculopathy, pulmonary haemorrhage, nephropathy (2-5 points) 4. Obstetric - pre-eclampsia, >/=3 consecutive miscarriages, fetal death (1-4 points) 5. Cardiac valve - thickening, vegetation (2-4 points) 6. Thrombocytopaenia (2 points) Laboratory: 1. Lupus anticoagulant +ve (1 point if one off, 4 points if persistent) 2. Anticardiolipin and anti beta2 microglobulin (up to 7 points)

Answer 682

Avoid smoking, OCP Treat HTN, hypercholesterolaemia/TG Low dose aspirin

Answer 683

Arthralgias, myalgias Cytopaenias Urticaria Rash, itch Lupus Psoriasis SJS/TEN Photosensitivity Altered taste Hedache GI - hepatitis

Answer 684

Anorexia Nausea, vomiting Headache, confusion, dizziness, fatigue Menstrual irregularities Photosensitivity Lupus SJS/TEN Altered taste Blurred vision

Answer 685

Cytopaenias Headache URTI Insomnia, somnolence Peripheral neuropathy Nausea, vomiting, diarrhoea, abdo pain Hepatitis, pancreatitis Gynaecomastia Impotence Heart failure HTN Pulmonary oedema EM AGEP SJS, TEN Urticaria, angioedema LCV

Answer 686

Itraconazole - Cat C Griseofulvin - Cat D Fluconazole - Cat D Terbinafine - Cat B1

Answer 687

Hypersensitivity Active liver disease CHF/ventricular dysfunction Anti-arrhythmics

Answer 688

200mg BD 1 week each month 3-4 months 200mg OD to BD for 1 week

Answer 689

Huff N Huff FAG Headache, dizziness Nausea, vomiting, abdominal pain, diarrhea Hyperkaelemia – muscle cramps, weakness; hyponatremia Feminisation of male fetus Agranulocytosis – elderly, renal/hepatic impairment Gynaecomastia, breast tenderness, menstrual irregularities, decreased libido Spiral stair case - Granite - agranulocytosis - Cost a fortune - many Ks - hyperkalaemia - Quite feminine - Makes you get a headache, dizzy, nausea, vomit - Difficult with gynaecomastia

Answer 690

Aldosterone antagonist Competitively inhibits androgen receptors, inhibits androgen synthesis Hirsutiusm FPHL Acne

Answer 691

Renal impairment Hyperkalaemia Personal or Family history of breast/ovarian/uterine cancer Abnormal uterine bleeding Drug interctions - ACE-I/ARB, NSAIDs Not pregnant or lactating – B3 feminises male fetus

Answer 692

Anti-androgen – competitively bind androgen receptors In OCPs - Diane, Estelle, Brenda – 2mg daily Or 25-50mg daily 10 days at start of cycle, given with OCP same time

Answer 693

Spironolactone analogue Yaz/Yasmin 3mg = 25mg spironolactone

Answer 694

2012 revised International Chapel Hill Consensus Conference Nomenclature system

Answer 695

vessel wall damage is directly mediated by neutrophils rather than by immune complex deposition “pauci-immune”

Answer 696

Neutrophilic infiltrate Leukoctyoclasia (production of neutrophil nuclear dust) Extravasated erythrocytes Fibrinoid necrosis of blood vessel walls

Answer 697

Pernio PLEVA Resolving LCV Rickettsial, viral infections AICTD, lupus, relapsing polychondritis Behcets LTA

Answer 698

Drugs (10-15%) Infections (15-20%) - bacterial (strep, mycobacteria, septic vasculitis), viral (URTI, hep, HIV, parvovirus B19) AICTD (15-20%) - RA, SLE, Sjogrens, IBD Malignancies (2-5%), most often haem Idiopathic (~50%)

Answer 699

Antibiotics - beta lactams, sulfonamides, tetracyclines, macrolides Retinoids OCP Allopurinol Beta blockers GCSF Anti-epileptics Frusemide Thiazides ACEi TNFs

Answer 700

90% spontaneous resolution weeks - months 10% may be more chronic or recurrent over a couple of years More likely to be chronic if arthralgias and cryoglobulinaemia and absence of fever (/also depends on underlying cause)

Answer 701

Minocycline Anti TNFs Levamisole Propylthiouracil, crabimazole Hydralazine

Answer 702

Arthritis (knees and ankles most common, 60-75% cases, transient migratory and non destructive) Renal (40-50%) Abdominal pain, melena (50-75%) - intestinal ischaemia Pulmonary haemorrhage - rare In boys, orchitis is a rare manifestation Fevers Urinalysis, UPCR, UACR, UECs, BP - if normal, repeat every 2 weeks for 1 month, then every 2 months for 6-12 months, then annual - if abnormal, refer renal FBE, if anaemia then FOBT, if FOBT +ve then refer for scopes

Answer 703

Elevated serum IgA can indicate higher risk Solid organ Mucosal, lungs Older age >60 and no other known trigger

Answer 704

Risk factors nephritis: age >8 years at onset, abdominal pain, and recurrent disease Poor prognostic factors for long term renal impairment: renal failure at the time of onset, nephrotic syndrome, hypertension, and decreased factor XIII activity 1-3% kids 30% adults

Answer 705

Usually 4 and 24 months of age Abrupt onset, fever 45%, otherwise well Annular, medallion, circular, or targetoid purpuric plaques on the face and extremities Tender, non-pitting oedema of acral sites Triggers (75%): infection, drug exposure, or immunization Spontaneous resolution within 1 to 3 weeks Mucosal and visceral involvement is rare - oral petechiae, conjunctival injection, abdominal pain, arthralgias, glomerulonephritis, and intussusception (<1%)

Answer 706

CHADI Complement deficiency Haem malignancy Autoimmune - SLE, Sjogrens Drugs - fluoxetine, diltiazem, cimetidine Infections - Borrelia, EBV GRAPE - systemic symptoms/ other features GI symptoms Renal - GN Arthritis, angioedema Pulmonary - COPD like Eyes - uveitis, episcleritis, conjunctivitis

Answer 707

2 major criteria: urticaria for 6 months + hypocomplementaemia Plus 2 or more minor criteria: (1) vasculitis on skin biopsy (2) arthralgia or arthritis (3) uveitis or episcleritis (4) glomerulonephritis (5) recurrent abdominal pain (6) positive C1q precipitin test with a low C1q level UH CARVE (2 major, 2 of 6 minor) Urticaria >6 months Hypocomplimentaemia C1q Abdo pain, arthralgia Renal - GN Vasculitis on bx Eye involvement

Answer 708

Elevated ESR Low C3/4 +ve ANA presence of anti-C1q precipitin depressed C1q levels

Answer 709

lime lemon fig parsley celery (furocoumarins)

Answer 710

Infections: - β-hemolytic streptococci, HBV, HIV, tuberculosis, and syphilis Autoimmune disease/ inflammatory: - granulomatosis with polyangiitis (Wegener granulomatosis) - IBD - Coeliac disease - Relapsing polychondritis - SLE - RA Benign and malignant haem disorders: - especially IgA monoclonal gammopathy - myelodysplasia, myeloproliferative disorders, and hairy cell leukemia Other systems Can get arthralgias Ocular disease - peripheral keratitis, nodular scleritis, panuveitis, and blindness

Answer 711

LCV, neutrophilic infiltrate, eos perivascular, concentric, or storiform fibrosis

Answer 712

Dapsone Intralesional corticosteroids NSAIDs Niacinamide Tetracyclines Colchicine

Answer 713

Type I Vascular occlusion (microvascular occlusion without vasculitis) Monoclonal IgM > IgG Clinical manifestations: Raynaud phenomenon, retiform purpura, gangrene, acrocyanosis Associations: plasma cell dyscrasias, lymphoproliferative disorders Type II, III “mixed cryoglobulins” Vasculitis Monoclonal IgM (>IgG) against polyclonal IgG; polyclonal IgM against polyclonal IgG Associations: HCV ≫ HBV, HIV, EBV, CMV, autoimmune connective tissue diseases (e.g. rheumatoid arthritis), lymphoproliferative disorders (e.g. B-cell non-Hodgkin lymphoma, CLL) Clinical manifestations: palpable purpura, arthralgias, peripheral neuropathy, glomerulonephritis

Answer 714

LCV DIF - granular deposits consisting predominantly of IgM and C3 in a vascular pattern are observed in the papillary dermis

Answer 715

Treat underlying cause interferon-α plus ribavirin Cyclophosphamide Prednisolone Rituximab Plasma exchange

Answer 716

proteinase 3 (PR3)

Answer 717

myeloperoxidase (MPO)

Answer 718

Palpable purpura, erythematous macules, urticarial or purpuric plaques, livedo racemosa, ulcers, splinter haemorrhage Renal - glomerulonephritis (crescentic necrotising) Neurologic - mononeuritis multiplex Pulmonary - capillaritis/haemorrhage, alveolar haemorrhage fever, weight loss, arthralgias, myalgias Associations: IE, meds, malignancy

Answer 719

Life or organ threatening: High dose CS (50-75mg/day) Rituximab Or cyclophosphamide - avacopan can be used to reduce CS exposure - plasma exchange cann be considered if rapidly progressing GN If not life threatening: MTX or MMF could be used in place of ritux Remission maintenance: Ritux MTX or AZA 24-48 months Mepolizumab can be an option for relapsing and not life or organ threatening EGPA Think of bactrim PJP prophylaxis

Answer 720

Skin: Palpable purpura Subcutaneous nodules PG-like ulcers PGND - palisaded neutrophilic and granulomatous dermatitis - umbilicated, crusted papulonodules on extensor surfaces (elbows) or face Friable, micropapular gingivae (strawberry gums) oral ulcers Upper respiratory: nasal ulcer saddle nose acute hearing loss chronic sinusitis/otitis/mastoiditis subglottic stenosis recurrent epistaxis nasal septal perforation Lungs: nodules, fixed infiltrates, cavities dyspnea, cough, hemoptysis, or pleuritis Renal - GN Ocular - proptosis, scleritis

Answer 721

Skin: Palpable purpura PNGD (papulonecrotic lesions) Subcutaneous nodules Urticarial plaques Livedo racemosa, retiform purpura, ulcers Upper respiratory: paranasal sinusitis, allergic rhinitis, nasal polyps Pulmonary: asthma, non-fixed infiltrate Haematologic: peripheral eosinophilia, elevated IgE Neurologic: mono or polyneuropathy, mononeuritis multiplex Cardiac: myo or pericarditis (cardiomyopathy) - leading cause of death Triggers: vaccination, desensitization therapy, leukotriene inhibitors, omalizumab and rapid discontinuation of corticosteroids

Answer 722

Segmental vasculitis of predominantly medium-sized arteries palpable purpura, livedo racemosa, retiform purpura, ulcers, subcutaneous nodules, or peripheral gangrene fever, weight loss, arthralgias, myalgias paresthesias (mononeuritis multiplex) abdominal pain orchitis renovascular hypertension and renal failure (not GN) shortness of breath (due to CCF)

Answer 723

Treat underlying triggers Colchicine Systemic steroids Cyclophosphamide AZA IVIG Dapsone HCQ MTX Plasmapheresis Pentoxifylline TNF alpha If digital necrosis: prostaglandins or calcium channel blockers If antiphospholipids: anticoagulants Often chronic, relapsing 6 monthly surveillance for progression to systemic PAN (rare <1% but often takes time >5years)

Answer 724

Segmental necrotising vasculitis of predominantly medium-sized arteries DIF may show deposits of C3, IgM, and fibrin within or around vessel walls

Answer 725

Infections: Hep B Hep C Strep Parvovirus B19 HIV Inflammatory diseases: IBD SLE familial Mediterranean fever Malignancies (especially hairy cell leukemia) Medications: Minocycline Empagliflozin, gemcitabine Genes: adenosine deaminase 2 (ADA2) deficiency

Answer 726

MPA: segmental necrotizing vasculitis of the smallest blood vessels (capillaries, venules and arterioles) and, less often, a vasculitis of small and/or medium-sized arteries GPA: Granulomatous inflammation Vasculitis of small +/- medium sized vessels EGPA: Extravascular eosinophils Granulomatous inflammation Extravascular granulomas Vasculitis of small +/- medium sized vessels

Answer 727

cutaneous ulcers, primarily of the frontolateral scalp and tongue tenderness along the course of the temporal artery visual disturbances muscle weakness of polymyalgia rheumatica USS, MRI, biopsy tocilizumab (IL6)

Answer 728

Lupus LP Sarcoid, GA Panniculitis PMLE, SU, PCT, DM Urticarial vasculitis Jessners lymphocytic infiltrate CI: 'PR HLM' Pregnancy/lactation (catt D), PsO Retinal/visual field changes Haem - severe blood dyscrasia Hypersensitivity Liver (significant hepatic dysfunction) Myaesthenia gravis or significant neurological disorder Interactions: Smoking Increase levels antimalarials: H2 antihistamines Decrease levels: PPI, antacids QT prolongation – macrolides Impairs activity of anticonvulsants

Answer 729

OH GCN 'OH Girl you Classy Now' Ocular - reversible and irreversible Reversible - halos, photophobia, diplopia, blurred vision Irreversible - bull's eye maculopathy, central scotoma, true retinopathy Haem Agranulocytosis Pancytopaenia GI N/V/D Transaminitis Cutaneous Blue grey hyperpigmentation PsO Hypersensitivity - morbilliform, lichenoid, eczematous, urticaria Bleaching hair roots Neuromuscular Irritability, mood swings, psychoosis Headache, vertigo, tinnitus Seizures Muscle weakness

Answer 730

Daily dose >400mg >5mg/kg/day >5-10 years Cumulative dose >1000g Hx of retinopathy or maculopathy Renal or liver dysfunction Use of tamoxifen Elderly

Answer 731

Erythema nodosum leprosum (Type 2 reaction) Prurigo nodularis Lupus, GVHD, sarcoidosis Neutrophilic dermatoses – Behcet’s AIDS-related KS Actinic prurigo

Answer 732

JAK 1 - abrocitinib, upadacitinib JAK 1/2 - barictinib, ruxolitinib JAK 1/3 - tofacitinib

Answer 733

Absolute: drug allergy, pregnancy (cat D), lactation, bone marrow suppression, history bladder cancer Relative: infection, renal/hepatic impairment

Answer 734

Oral: 1-2mg/kg/day IV pulse: 10-15mg/kg 4weekly FBC, EUC, LFT, urinalysis – weekly 2-3m Urine cytology 6monthly

Answer 735

Absolute: Hypersensitivity, childbearing potential, men having intercourse with women of childbearing potential, peripheral neuropathy (preg cat X) Relative: Cardiac, renal, hepatic, GI impairment, hypothyroidism

Answer 736

Thalma and Louise Chorus: Riding in a Getaway Car; there were sirens in the Beat of your Heart Teratogenicity Leukopaenia (and other haem - thrombocytopaenia) Clots/VTE Rash (SJS/TEN) GI - nausea, constipation CNS - depression, drowsiness, dizziness, hearing loss, peripheral neuropathy Bradycardia Hypothyroidism (rare: hepatotoxicity, pneumonitis)

Answer 737

50-300mg nocte Need written consent Baseline neuro exam, FBE, UEC, LFTs Best to take at night Stop if signs of VTE, peripheral neuropathy Monitor TFTs, HR, symptoms of neuropathy FBE Neuro exam Monthly for 3 months, then 3 monthly

Answer 738

Schauman bodies - round, blue, calcified, laminated inclusions Basophilic Residuum of lysosomal organelles Asteroid bodies - stellate, intracytoplasmic eosinophilic inclusions Engulfed collagen

Answer 739

Dimethylglioxime

Answer 740

Focal, visible excess sweating Present for at least 6 months No secondary causes And at least 2 of the following: - Bilateral and symmetric - Impairs activities of daily life - At least 1 ep per week - Age of onset <25 - Positive fam hx - Stops during sleep Sweat Sam Smith WAS FAB Excess sweating Six months No Secondary causes Weekly Activities daily life impaired Sleeping Family history Age <25 Bilateral and symmetrical

Answer 741

Hyperhidrosis Catch My Sweat And Catch my Drips Hypothalamic* Cortical* Medullary* Spinal* Autonomic Compensatory Damage

Answer 742

DINE CPG Diabetes, drugs Infections - TB, malaria, HIV Neoplasms - lymphoma, leukaemia Neurological - stroke, spinal injury, Parkinsons Endocrine - menopause, hyperthyroidism, phaeochromocytosis, carcinoid Compensatory - diabetes PPK Genoderm - CIE, EI, EBS

Answer 743

SSSO AAAAAA SSRIs Sulfonylureas Sildenafil Opioids Anti-Alzheimer drugs - cholinesterase inhibitors Antipsychotics Adrenomimetics - adrenaline, noradrenaline, dopamine ACE inhibitors Amlodipine Amphetamines Other: pentoxyfilline, tramadol, antipyretics, insulin

Answer 744

Differentiate between primary and secondary on history Investigations for secondary: FBE, UEC ESR, CRP TFTs BSL, HbA1c QFG CXR ANA Urinary catecholamines Then starch–iodine or quinizarin methods (colorimetric) - iodine solution (e.g. 3.5% in alcohol) applied to clean, shaved skin, allowed to dry - Starch powder (e.g. cornstarch) brushed onto area - Sites with sweating turn blue–black Gravimetric weighing filter paper before and after application to the skin Evaporative (device) Infrared thermography

Answer 745

TOP aluminum chloride hexahydrate 20% - 3-5 consecutive nights, then 1-2x per week as needed topical glycopyrrolate (0.5-2%) Oral: α2-adrenergic agonists e.g. clonidine beta blockers e.g. propranolol anticholinergics: Oxybutynin 5 mg BD (TDS, up to 20mg daily) Glycopyrrolate 1mg BD Propanthaline 15mg TDS (max 75mg per day) Botulinum toxin A - prevents release of acetylcholine from cholinergic neurons Iontophoresis Surgical: dissection, sympathectomy

Answer 746

Pregnant Metal implants - braces, dental band Pacemaker Cardiac arrhythmia Glaucoma Epilepsy

Answer 747

Pin, infection, bruise, bleed (multiple small needles) Takes 2 weeks to take effect Human albumin May not work Is temporary Financial consent Overall safe - no risk of poisoning

Answer 748

Hyperkeratosis Epidermal rete ridges often encircle dilated vessels Dilated vessels in superficial dermiss Thrombi

Answer 749

MDT Genetic counselling Enzyme replacement therapy with recombinant alpha galactosidase A Gabapentin, carbamazepine for acroparaesthesia Angiokeratomas - laser, cryo, electrosurgery Address cardiac risk factors ACEi for renal disease

Answer 750

Dense dermal infiltrate of neutrophils, lymphocytes, plasma cells, eosinophils Vasculitis (focal) Grenz zone Fibrosis

Answer 751

Difficult to treat TCS/CNI ILKA 2.5-5 Dapsone HCQ PUVA Laser (ablative, PDL) Excision, cryosurgery, dermabrasion, electrosurgery

Answer 752

Malignancy - esp T cell lymphoma and gastric carcinoma Also: Hodgkin disease, B cell lymphoma, CLL, AML, lung, colon, hepatocellular, prostate, renal, laryngeal Infections (HIV, hep C) Also parasites - strongyloides Fungi - dermatophytes Drugs Frusemide Rantidine Isoniazid Aspirin Dideoxyinosine Atopic dermatitis

Answer 753

Other name: epithelioid haemangioma Can be polypoid Dermal Vascular proliferation "Hobnail" endothelial cells (protruding into lumina) Endothelial vacuoles Eosinophils (many), plasma cells, lymphocytes

Answer 754

Granuloma faciale Rosai-Dorfman disease Kimura disease Angiolymphoid hyperplasia with eosinophilia

Answer 755

Oral CS Phototherapy +/- systemic retinoid eg acitretin CsA, AZA

Answer 756

Diffuse dermal infiltrate eosinophils and histiocytes (sometimes subcutis or deeper) Foci of amorphous/granular material ("flame figures") - reflect degranulation of eosinophils May have spongiosis in epidermis

Answer 757

Eos around hair follicles and sebaceous glands Eosinophilic and lymphocytic dermal infiltrate Infundibular eosinophilic pustules +/- spongiosis

Answer 758

Primary, secondary, idiopathic or hereditary Primary - neoplasm including eosinophilic leukaemia Secondary - inflammatory hypereosinophilia Mucocutaneous >50% Cardiac - eosinophilic endomyocardial disease Diagnosis - Eos >1.5 at least 1 month apart or tissue eosinophilia - Organ damage due to hypereosinophilia - No other cause for hypereosinophilia (parasitess, allergy) Treatment Imatinib (if primary) PO steroids Interferon Anti IL5 monoclonal antibody Dapsone Treat underlying disease if secondary

Answer 759

TCS Antihistamines - second generation H1 - can add H2 PUVA, nbUVB Oral cromolyn sodium - mast cell stabiliser Oral CS Omalizumab Cladribine Imatinib (codon 816 KIT mutation typically does not respond to this) Other chemotherapy, radiotherapy Splenectomy Epipen Calcium, vit D PPI

Answer 760

MAST C Mast cells >15 aggregated bone marrow or extracutaneous Activating c kit mutation Spindled or atypical mast cells >25% Tryptase >20 CD2/25 expression on mast cells

Answer 761

Indolent Smoldering Aggressive Systemic with associated haematological malignancy Mast cell leukaemia Mast cell sarcoma

Answer 762

Pruritus, flushing, abdo pain, diarrhoea, palpitations, dizziness, syncope Fever, night sweats, malaise, weight loss, bone pain, epigastric distress, problems with mentation Extracutaneous: Bone - Boney lesions, demineralisation, osteosclerosis, osteoporosis Bone marrow Splenomegaly Lymph node enlargement GI - abdo pain, diarrhoea, nausea, vomiting. Also higher risk gastritis and peptic ulcers Hepatomegaly CNS - irritability, fatigue, headache, poor attention span, short-term memory

Answer 763

Mast cells (abundant eosinophilic cytoplasm, with granules, large pale nuclei) 15 in high power field is usual cut off Melanin hyperpigmentation Stains: Toluidine blue Leder Giemsa Mast cell tryptase IHC: CD 117 (KIT)

Answer 764

DR MASTO Dextromethorphan (cough suppressant), dextran in some IV solutions Radiocontrast (iodine-based) M (N) for NSAIDs Anticholinergics, alcohol, aspirin Suxamethonium and other systemic anaesthetics (monitor for at least 24 hrs, can get delayed anaphylaxis) Tasty things - alcohol, spicy foods, hot beverages Opioids CHEIF - cold, heat, exercise, insects, friction

Answer 765

* Acne, dissecting cellulitis, pilonidal disease * Pyoderma * Depression, GAD, suicide * Smoking, substance use disorder * PCOS, obesity, dyslipidaemia, diabetes, metabolic syndrome, HTN, CVD * IBD, spondyloarthritis * Sexual dysfunction

Answer 766

AD. Gamma secretase. 3 genes - NCSTN, PSEN1, PSENEN. Deficiency in Notch 1

Answer 767

Regular - recurrent lesions, typical locations Frictional furuncle - regular PLUS multiple deep nodules and abscesses on friction sites (abdomen, thighs, buttocks) often overweight Scarring folliculitis - regular PLUS pustules, cysts, superficial nodules, double headed comedones. Significant scarring often overweight + smoke Conglobata - cyst formation and acne conglobata lesions back and face Usually familial (gamma secretase) often male and not overweight Syndromic - PASH, PAPASH

Answer 768

Hurley Sartorius HS PGA HiSCR DLQI IHS4

Answer 769

ADIOS PAPI Acne, dissecting cellulitis, pilonidal disease Depression, anxiety, suicidal ideation Diabetes, up to 30% pts. 3x increased risk, Dyslipidaemia IBD. 2x increased risk Crohn's, 1.5x increased risk UC Obesity, ~75% pts Smoking, Substance use disorder, Scc (more common in men. Anal/gluteal area). Sexual dysfunction SAPHO, PASH, PAPASH, PAPA PCOS, ~10% pts, 3x increased risk Arthritis, up to 50% pts spondyloarthritis Pyoderma gangrenosum Insulin resistance, metabolic syndrome. HTN, CVD

Answer 770

Anaemia Secondary amyloidosis Lymphoedema Fistulae to bladder, urethra, peritoneum, rectum SCC - Marjolin ulcer. Most common gluteal and perineal Infection Scar Pain Pyogenic granuloma Substance abuse

Answer 771

Vulval pain related to specific cause - Infection (candida, herpes) - Inflammation (LP, LS, immunobullous) - Neoplastic (Pagets, SCC) - Neurologic (spinal nerve compression) Vulvodynia - Generalised vs localised - Provoked vs unprovoked (or mixed)

Answer 772

Menstruation in 25-50% OCP

Answer 773

Mother - Graves disease Baby - SGA - Prematurity - Mild transient lesions of pemphigoid gestationis in 10% due to passive transfer of maternal antibodies, resolves in days to weeks

Answer 774

Yes and earlier May not occur in first pregnancy

Answer 775

Spongiosis Subepidermal blister Papillary dermal oedema Mixed cellular infiltrate dermis, with eos DIF - linear C3 (100%) and IgG (sometimes) BMZ

Answer 776

General measures - soap free wash, emollient - menthol - lance blisters - dressings - wet dressings Potent TCS Antihistamines - dexchlorpheniramine/polaramine nocte - loratadine B1, cetirizine B2 If severe, and with liaison with obstetrician, - pred 0.5mg/kg weaning - first trim esp weeks 8-11 and high doses, risk cleft lip/palate - monitor BSLs, gestational diabetes, BP (PE) Other options if severe/recalcitrant: Plasmapheresis IVIG CsA Post delivery can manage as per BP depending on breastfeeding

Answer 777

Usually primiparous Multiple gestations higher risk Does not recur No risks/complications

Answer 778

multiple gestation pregnancies past hep c infection genetically linked elevated total serum bile acids jaundice in 10% LFTs may be normal in 30% recurs in 45-70% recurs with OCP fetal risk premature births (20–60%) intrapartum fetal distress (20–30%; e.g. meconium staining of amniotic fluid, abnormal fetal heart rate) fetal loss (1–2%) mother small risk steatorrhea and vitamin K deficiency and therefore PPH treatment - ursodeoxycholic acid

Answer 779

Atopic individuals For 80% it is their first eczema 75% occur before 3rd trimester Elevated IgE in 70% 2/3rds eczematous at atopic sites - face, neck, flexures 1/3rd papular at trunk and extremities No risks/complications Commonly recurs

Answer 780

Pigment - melasma - hyperpigmentation - linear nigra, areolae Hair - hirsutism - postpartum TE Nail - brittleness, onycholysis, subungual hyperkeratosis Striae (gravidarum) Vascular - pyogenic granulomas - varicosities - spider angiomas - palmar erythema - non-pitting oedema - haemorrhoids

Answer 781

Incubation - weeks - 2 months for cutaneous - longer (3 - 9 months) for mucocutaneous

Answer 782

cutaneous, disseminated cutaneous, diffuse cutaneous, mucocutaneous/mucosal, and visceral leishmaniasis

Answer 783

Leishmania major L tropica L infantum L donovani L aethiopica

Answer 784

L braziliensis L mexicana L chagasi L amazonesis

Answer 785

L infantum L donovani L chagasi

Answer 786

L braziliensis L panamensis L guyanensis

Answer 787

L aethiopica L amazonesis

Answer 788

L braziliensis L amazonesis

Answer 789

Epidermis - may have ulceration, pseudoepitheliomatous hyperplasia Amastigotes in macrophages mixed inflammatory infiltrate with histiocytes, lymphocytes, plasma cells, and neutrophils Stains - Giemsa, CD1a, Wright If long standing - tuberculoid granulomas with caseation necrosis

Answer 790

Prevention of sand fly bites - DEET-based repellants, bed netting, permethrin-treated clothing, and sleeping in minimal-risk areas Top Paramomycin 15% (10 to 20 days) Antimonials: IV, IM or IL sodium stibogluconate or meglumine antimoniate; 20 mg/kg/day IV or IM × 20 days; IL - 0.2–5 ml (5 sites/lesion intradermally for 0.1 ml/cm2 until blanched) every 3–21 days for 5–8 sessions or until healing Miltefosine 50mg BD if weight is 30-44kg TDS if weight >/=45kg 28 days Oral fluconazole 6 weeks Liposomal amphotericin B - 3 mg/kg/day IV on days 1–5 + 10 or days 1–7 Pentamidine Other: PDT Cryotherapy

Answer 791

Zoonotic - L major Anthroponotic - L tropica Recidivans - chronic, destructive, recurrent Lupoid - lupus vulgaris like

Answer 792

tissue impression smears (touch preparations) - amastigotes smears obtained by dermal scraping or needle aspiration of skin lesions - amastigotes culture and PCR biopsy or needle aspirate delayed skin reaction test (Montenegro skin test or Leishman reaction) - killed promastigotes injected intradermally - positive if a papule >5 mm in diameter forms at the site of inoculation after 48–72 hours serology - indirect immunofluorescence, ELISA, immunoprecipitation, and isoenzyme electrophoresis

Answer 793

Nematoda (roundworms) and Platyhelminthes (flatworms) Among flatworms: trematodes/flukes cestodes/tapeworms

Answer 794

Due to larvae of animal hookworms (intestinal nematodes) Ancylostoma braziliense Ancylostoma caninum Uncinaria stenocephala Bunostomum phlebotomum serpiginous and pruritic feet and buttocks rate of migration is 1–2 cm per day Ivermectin 12mg in adults, 200 mcg/kg in children Albendazole

Answer 795

autoinfection with Strongyloides stercoralis in immunocompetent individuals typically begins perianally rapidly extends, as quickly as 5–10 cm per day, onto the buttocks, thighs, and abdomen

Answer 796

Onchocerca volvulus - nematode subcutaneous nodules that contain adult worms (onchocercomas), a pruritic papular dermatitis, lichenification, and leukoderma hanging groin or elephantiasis of the genitalia sclerosing keratitis can lead to blindness Dx: Identification of microfilariae or worm in skin/histo Slit lamp eye exam “Mazzotti test” - 50 mg of diethylcarbamazine (DEC) and pruritic eruption in 15 mins PCR Treatment: Ivermectin Doxycycline Nodulectomy

Answer 797

Nematodes (roundworms) Most common Wuchereria bancrofti lymphangitis and orchitis lymphoedema and elephantiasis diagnosis: Detection of circulating filarial antigen demonstration of microfilariae in the blood, urine, or other body fluids and tissues identification of adult worms PCR treatment: Diethylcarbamazine 6 mg/kg/day for 12 days (targets microfilariae) - exclude other helminthic diseases before instituting therapy; because can get a severe reaction in patients who have loiasis or onchocerciasis Doxycycline (targets adult nematode) Antihistamines and corticosteroids elevation of the affected body part, compression stockings, treatment of secondary infections, and protection of the affected area from trauma

Answer 798

trematode (fluke) swimmers itch - areas of skin covered by clothing are spared treatment: Praziquantel

Answer 799

acquired mammalian meat allergy due to IgE antibodies directed against galactose-alpha-1,3-galactose (alpha-gal) acquired following tick bites Ixodes holocyclus in Australia delayed onset urticaria, angioedema, and gastrointestinal symptoms following red meat consumption anaphylaxis Ix: Alpha gal specific IgE (prick or intradermal test) Mx: Red meat avoidance Symptomatic - antihistamines, antiemetics, antidiarrhoeals, oral cromolyn solution, omalizumab, epipen void subsequent tick bites

Answer 800

Obligately intracellular gram negative bacteria Reside within arthropod (tick, flea, louse, mite) Symptoms and signs: Increased vascular permeability -> oedema, hypovolaemia, hypotension High grade fever, headache, myalgia, nausea, vomiting, abdo pain, tenderness Rash day 3-6 (macules then papules then petechia then purpura then cutaneous necrosis/gangrene) (also eschar, erythema and oedema at site of original tick bite) Cough, confusion, lethargy Lung - pulmonary oedema, ARDS, resp failure Brain - meningoencephalitis, coma AKI Low: plts, Hb, Na Deranged LFTs, UECs

Answer 801

Biopsy - histo: Lymphocytic vasculitis, LCV Extravasation RBCs Dermal or perivascular oedema Fibrin thrombi Lymphohistiocytic infiltrate PCR skin biopsy, IHC Serology is slow

Answer 802

Doxy 100mg BD Others: rifampicin 300mg BD, azithromycin 500mg daily *report to department of health

Answer 803

Regional lymphadenopathy Conjunctival hyperaemia Hearing loss

Answer 804

Bacteria: Coxiella Burnetti Tick: Amblyomma triguttatum (ornate kangaroo tick) or Ixodes holocyclus Transient punctiform rashes, purpuric or maculopapular Endocarditis Fever + pneumonia + hepatitis EN

Answer 805

Bacteria: Rickettsiae australis Tick: Ixodes holocyclus or tasmani

Answer 806

Paralysis Anaphylaxis Mammalian meat allergy Deafness (if in auditory canal)

Answer 807

Permethrin treated clothing Topical repellent - DEET, picardin, tick repellents Extra caution Sept - April Long sleeves, pants tucked into boots Inspect skin visually and with hands Shower, hot water washing cycle laundry

Answer 808

Permethrin cream if small If large (>2mm) - freezing spray e.g. medi freeze tick off Fine tipped forceps with professional Grasp as close to skin as possible Gentle firm pull directly upwards/perpendicular to skin Do not squeeze tick body Clean with alcohol or soap water Dispose of tick - alcohol. sealed bag/container, tape

Answer 809

Acute - erythematous macule, papule, plaque - necrotic ulceration/eschar - vesiculobullous lesions - oedema - papular urticaria - papulo-pustulr eruption - secondary infections - cellulitis, impetigo, echthyma - bruising, purpura - enlarged or tender LNs - EN (rare) Chronic - tick bite granulomas - lichenification - PIH - keloid Also exacerbation or unveiling of underlying dermatosis such as AD or DH

Answer 810

FIXED PACE Fanconi's anaemia Incontinentia pigmenti Xeroderma pigmentosum Erythema ab igne Dowling degos Dyskeratosis congenita Drugs Prurigo pigmentosa Amyloid (macular) Addisons Confluent and reticullated papillomatosis EB simplex with mottling Naegeli-Franceschetti-Jadassohn syndrome

Answer 811

NED PLANKS Neoplastic - met melanoma (with melanosis), ACTH-secreting tumour Endocrine - carcinoid, Addison's, hyperthyrooidism, phaeochromocytoma Drugs PCT Liver disease - Wilsons, haemochromatosis Anaemia (Fanconi, IDA, haemolytic) Nutritional deficiency (pellagra, B12, Kwashiorkor) Kidney failure

Answer 812

DZ CHAMP CHAMP Diltiazem Zidovudine Chemotherapeutics (bleomycin, MTX, 5-FU, imatinib, hydroxyurea, cyclophosphamide) Hydroquinone Antimalarials Minocycline Psychotropics Clofazamine Hormones - OCP, MSH Amiodarone Metals Psoralens

Answer 813

Tinted sunscreens (iron oxide) (need visible light protection) Camouflage Review OCP Topical 4% hydroquinone Triple combination cream - different versions - 4% hydroquinone, 0.05% tretinooin, 0.01% fluocinolone acetonide Azelaic acid 15-20% Kojic acid 1-2% Ascorbic acid 5-25% Niacinamide 5% Arbutin Bakuchiol 0.5% Thiamidol 0.1-0.2% Topical tranexamic acid 2-3% Tranexamic acid 500-750mg daily (in split dose i.e. 250mg BD) Polypodium leucotomos extract (For refractory cases only): Chemical peels Microneedling QS nanosecond or picosecond laser IPL (not for darker skin types) Nonablative fractionated laser in lighter skin types

Answer 814

centrofacial (most common) - forehead, cheeks, nose, upper lip (sparing the philtrum and nasolabial folds), and chin malar, affecting the cheeks and nose mandibular, along the jawline Less common: extensor aspect of the forearms and mid upper chest

Answer 815

Increased melanin in epidermis, particularly in basal layer (hypertrophic, pendulous melanocytes) Increased melanophages in upper dermis Epidermal melanocytes normal to slightly increased in number

Answer 816

Haemosiderin - Pearls Prussian Blue; Melanin - schmorl's stain

Answer 817

Sun exposure Pregnancy Oral contraceptives other medications (e.g. phenytoin, phototoxic drugs) autoimmune thyroid disease

Answer 818

Drug induced hyperpigmentation Riehls pigmented contact dermatitis LPP PIH Hori naevus Exogenous ochronosis

Answer 819

Tyrosinase inhibitor CI: Pregnancy, breastfeeding Course: Max 3-6 months continuously SEs: Irritation ACD Erythema Inflammation Xeroderma Stinging Ochronosis Colloid milium Hypopigmentation Leukoderma at distant sites (stop and then start TCS)

Answer 820

Isotretinoin Dapsone Doxycyline in EDP Steroids in LPP Clofazamine in EDP nbUVB

Answer 821

Aetiology unknown Gray–brown to blue–gray macules and patches in a symmetric distribution >5cm generally Trunk --> neck --> proximal upper extremities --> face (sometimes) Early lesions may have a thin, raised, erythematous border (in EDP) Slowly progresses Typically does not regress in adults, but spontaneous resolution can happen in children (~2/3rds)

Answer 822

Lichenoid perivascular infiltrate /Dermal usually perivascular lymphocytic infiltrate May or may not have interface dermatitis Pigment incontinence - melanophages IHC - ICAM-1 CD69, CD94

Answer 823

Smaller macules compared with EDP 0.5-2cm Asymptomatic Resolves in months to years Usually adolescents and young adults Neck, proximal extremities, trunk Histo: Hyperpigmentation basal layer, prominent dermal melanophages, without basal layer damage or lichenoid inflammatory infiltrate My have papillomatosis

Answer 824

Head and neck most commonly involved, followed by flexures Neck --> jawline --> temples, lateral forehead May or may not also have LP (minority) Histology similar to EDP

Answer 825

Type 1 - in sCars (no time/dose dependent relationship) Type 2 - sHins Type 3 - sUn exposed areas (tends to persist)

Answer 826

FILL X CoMB Flagellate pigmentation (bleomycin, shiitake mushroom dermatitis) Focal dermal hypoplasia (Goltz) IP Lichen planus Linear nevoid hypermelanosis X linked reticulate pigmentary disorder Conradi Hunnerman Happle Morpheoa, linear atrophoderma of Moulin Blashkitis

Answer 827

V-shape on the posterior midline S-shape on the abdomen Spirals on the posterior scalp

Answer 828

Lentinan, a thermolabile toxin in shiitake mushrooms

Answer 829

IKBKG/NEMO X linked vesicles (stage 1), verrucous lesions (stage 2), hyperpigmentation (stage 3) and, eventually, hypopigmentation (stage 4)

Answer 830

Hyperpigmentation in whorls and streaks following the lines of Blaschko Usually within 1st year of life Systemic features in minority neurologic, musculoskeletal, or (less often) cardiac in nature Histo: Increased melanin within the epidermis (in basal keratinocytes) few dermal melanophages Ddx: IP Epidermal naevus

Answer 831

perivascular and interstitial neutrophil-dominant mixed infiltrate dyskeratotic keratinocytes

Answer 832

= Systemic-onset juvenile idiopathic arthritis Avge age onset 6 years High spiking fevers Lymphadenopathy Hepatosplenomegaly Arthritis Transient cutaneous eruptions of erythematous macules and papules - accompanying fevers, evanescent - Koebner phenomena - Non-pruritic - axillae and waist - periorbital edema and erythema Other features: Leucocytosis with neutrophilia Anaemia (often severe) Thrombocytosis, elevated ESR, elevated ferritin and CRP Polyclonal gammopathy Elevated liver enzymes Occasionally: - Myocarditis - Pneumonitis - Anterior uveitis (<1%) - CNS involvement - Macrophage activation syndrome (~10%) - Amyloidosis (rarely)

Answer 833

1. High episodic fevers daily for ≥2 weeks 2. Arthritis: symmetric polyarticular arthritis > oligoarticular arthritis (erosive in 20%) At least one of the following 1. evanescent eruption 2. generalized lymphadenopathy 3. hepatomegaly or splenomegaly 4. serositis

Answer 834

Occurs in 10–20% of patients treated with systemic bleomycin Flagellate (or circumscribed or diffuse) pigmentation Some have severe pruritus, some have none painful nodules on the fingers transverse melanonychia, also Beau's lines, onychomadesis, and onycholysis sclerodermoid changes verrucous plaques on the knees and elbows digital gangrene due to Raynaud phenomenon Histo: basal layer hypermelanosis spongiosis confined to the basal layer perivascular lymphohistiocytic infiltrate admixed with melanophages

Answer 835

~Half resolve spontaneously/completely ~Half have a chronic course with persistent arthritis, systemic complications such as macrophage activation syndrome, hepatitis, pericarditis, and rarely amyloidosis Treatment: NSAIDs Systemic corticosteroids MTX TNF alpha inhibitors AZA Thalidomide IL-1 antagonists (e.g. anakinra, rilonacept, canakinumab) IL-6 antagonists (e.g. tocilizumab)

Answer 836

erythematous papules and papulovesicles on the back, neck, and chest leaving macular reticulated hyperpigmention Rx: tetracyclines, dapsone

Answer 837

MDT (incl haem) Longitudinal surveillance of all mucosal surfaces - for SCCs within leukoplakia Sun and cigarette avoidance

Answer 838

AD Keratin 14 Reticulated hyperpigmentation beginning by 2 years, fades by adolescence PPK Hypohidrosis, heat intolerance dental anomalies, including early loss of teeth onychodystrophy

Answer 839

AD Keratin 14 Triad of: reticulated hyperpigmentation (persists) non-scarring alopecia onychodystrophy

Answer 840

POLA1 Reticulated hyperpigmentation along the lines of Blaschko in women and in a generalized pattern in men Systemic manifestations in men: Blonde unruly hair with a frontal upsweep Recurrent pneumonia and COPD Low birthweight, neonatal colitis, failure to thrive during infancy, seizures and hemiplegia, as well as gastroesophageal reflux, urethral strictures, and inguinal hernias Dental anomalies, hypohidrosis, xerosis, photophobia, corneal clouding, and skeletal changes such as delayed bone age and shortened metacarpals

Answer 841

AD Keratin 5 Reticulated hyperpigmentation in flexural sites Comedone-like lesions on the back and neck Pitted facial scars Rx: Topical hydroquinone, tretinoin, adapalene, and corticosteroids Erbium YAG

Answer 842

Dyschromatosis Symmetrica Hereditaria - dorsal aspects of the distal extremities, especially the hands and feet Dyschromatosis Universalis Hereditaria - generalised head, neck, extremities, and trunk

Answer 843

Noonan syndrome with multiple lentigines (LEOPARD) Carney Complex Peutz Jeghers

Answer 844

Physiologic pigmentation or racial pigmentation Smoker's melanosis Amalgam tattoo or pigmentation Melanotic macule or mucosal lentigines

Answer 845

Physiologic Iatrogenic/drug induced (zidovudine, antimalarials, chemotherapeutics) Inflammatory - LP Endocrinopathies - Addisons, cushings, hyperthyroid Benign melanocytic naevi Melanoma Bacterial infection - pseudomonas Fungal infection Haematoma Exogenous - silver nitrate

Answer 846

Longitudinal band wider proximally than distally Most common at thumb and great toe Width >2/3 nail plate Grey or black colour Irregularly pigmented lines Hutchinson's and micro Hutchinson's Nail dystrophy Granular pigmentation

Answer 847

retinoids, BRAF and EGFR inhibitors, trauma pregnancy - granuloma gravidarum

Answer 848

Advanced maternal age Female Caucasian Premature birth Placental abnormalities - placenta previa, pre-eclampsia

Answer 849

C+C, risk recurrence Surgery PDL Cryo Silver nitrate Imiquimod

Answer 850

Polypoid, epidermal colarette dilated interconnecting capillaries/vessels within the papillary dermis

Answer 851

ill-defined, often indurated, violaceous stain, plaque, or bulging mass can penetrate deep a/w KMP

Answer 852

Pink red plaques Neck (trunk or shoulders) 50% <5yo May have associated growth of lanugo hair or port-wine-like stain A/w Kasabach-Merritt phenomenon Histo: - tightly packed tufts (/multiple discrete lobules) of tiny capillaries distributed within the dermis and subcutis in a “cannonball” pattern - poorly defined - May have fibrotic dermal collagen - Negative for GLUT1 and Lewis Y antigen - platelet trapping can be confirmed with CD61 immunoreaction

Answer 853

Spindle cell proliferation Can be in fascicular fashion (schools of fish) Slit like spaces (containing erythrocytes) Promontory sign Sieve-like pattern of vascular spaces Plasma cells HHV8+ CD31, CD34 VEGFR-3 podoplanin eRG

Answer 854

Proliferation of blood vessels surrounded by glomus cells (monotonous cells with dense round nucleus and abundant pink cytoplasm) Pale stroma Positive staining for smooth muscle actin +/- desmin

Answer 855

Classic KS - older men, lower legs, Mediterranean and Middle European descent - slow growing - may progress to more multifocal/disseminated African endemic - nodular (more like classic KS), florid, infiltrative or lymphadenopathic (esp in children, poor prognosis) Iatrogenically immunocompromised - clinically similar to classic KS, depending on level of immunosuppression can be more aggressive HIV associated - Broad spectrum of clinical disease - single, disseminated, macules, plaques - Typically more widely distributed lesions - Trunk and midface (esp nose) common sites - Can get LN, lung and GI involvement (esp poor prognosis with lung)

Answer 856

If iatrogenic, reduce immunosuppression (or switch CNI to mTOR) If HIV, treat If superficial - cryotherapy, laser, photodynamic therapy, intralesional vinblastine or vincristine, topical imiquimod Surgery if solitary Radiation, chemotherapy

Answer 857

Bruise-like patch plaque or nodule on face or scalp 5 year survival <20% Risk factors: lymphoedema, radiotherapy, chemical exposures (vinyl chloride, thorium dioxide, arsenic, radium, and anabolic steroids) Histo: Poorly demarcated dissecting blood vessels with irregular branching Papillary projections into lumina Slit like spaces Spindled or epithelioid atypical endothelial cells Prominent extravasated erythrocytess Haemosiderin CD31, CD34, erg

Answer 858

Angiosarcoma secondary to lymphoedema aggressive form of cutaneous lymphangiosarcoma

Answer 859

- higher degree HLA disparity - donor/recipient gender disparity - CMV status of donor and host - matched unrelated greater risk than sibling donor - older age of recipient - source of HSCT (peripheral blood > BM > cord) - intensity of conditioning regimen used - GVHD prophylaxis regimen

Answer 860

Cholestatic heptatitis (transaminitis, bilirubin elevation) Morbiliform eruption Bloody diarrhoea May be thrombocytopaenic

Answer 861

MDT TCS Ruxolitinib Oral pred IV methylpred MMF TNF

Answer 862

SPILL FAKE VENOM Scleroderma/morphoea -like, SCLE -like, SCC Psoriasiform, pruritus, poikiloderma, pigmentation, papulosquamous Ichthyosis Lichen planus Lichen sclerosus Limb oedema, lungs - bronchiolitis obliterans Fasciitis (eosinophilic) Alopecia, angiomatosis, acral erythema Keratosis pilaris like Eczematous Vulvovaginal Exfoliative, eyes Nails Oral - sicca Mucocoele Milia

Answer 863

MDT (heam, oral mucosal, ophthalm, physicians) Skin care, sunprotection, skin cancer surveillance, avoid traum/friction TCS, Top CNI Physio Compression nbVB Mouth care - toothbrush, toothpaste and medicated rise/TCS/antifungal Eye drops, sunglasses 1st line - pred +/- CNI (CsA) 2nd line - CsA/TAC ECP MTOR inhibitor (sirolimus) MMF Imatinib Rituximab MTX Ibrutinib Ruxolitinib

Answer 864

Prayer sign, elbow extension, foot dorsiflexion, shoulder abduction

Answer 865

Lungs - SOB, cough (bronchiolitis obliterans) Heart - cardiomyopathy MSK - cramps, pains, weakness, swelling, tightness Neuropathy GIT - diarrhoea, ooesophgeal stenosis/strictures, transaminitis, exocrine pancreatic insufficiency Salivary glands - sicca syndrome Eyes - keratoconjunctivitis sicca, blepharitis, corneal erosions

Answer 866

NLD, livedoid vasculopathy, ulcers/PVD, raynaud's, PPD, GA Hypersensitivity (to methyl xanthines) Pregnancy, breastfeeding (cat C) Caution Bleeding Severe cardiac disease

Answer 867

Headache, dizziness Nausea, vomiting, GI discomfort Rare – angina, palpitations, bleeding

Answer 868

Antiviral, antiproliferative, immunoregulatory Indications: CTCL, HIV associated KS, B cell lymphoma, behcets, viral papillomas

Answer 869

Hypersensitivity pregnancy (cat D), lactation, organ transplant, cardiopulmonary disease, diabetes prone to ketoacidosis, depression, cytopenias, coagulopathies

Answer 870

Classic G Flu Can't Handle Some Rhabdo CNS: dizziness, confusion, memory loss, paresthesia, weakness GI: nausea, diarrhoea, anorexia, transamninitis Flu-like symptoms – common; fever, headache, myalgia, arthralgias CVS: hypotension, dysarrhythmia Haem: cytopenias Skin: dry skin, temporary hair loss Rhabdomyolysis

Answer 871

IgA deficiency CHF, renal failure (fluid overload) Cryoglobulins, RF (risk renal failure) Ix: Cardiopulmonary, renal and fluid status FBE UEC LFT IgA, Hep B/C, HIV, cryoglobulins, RF

Answer 872

Equivalents: Prednisolone 1mg Hydrocortisone 4mg (divide by 4 to get Pred dose) Dexamethasone 0.15mg (multiply by 7 to get Pred dose) 25mg pred 100mg hydrocortisone 4mg dexa 28mg pred

Answer 873

Systemic infection Live vaccination Precaution with: diabetes, HTN, CHF, psychosis CYP3A4 those inhibitors and inducers warfarin NSAIDs (PUD) preg cat A

Answer 874

Skin: Poor wound healing Ulcers Atrophy Striae Telangiectasia Bruising Steroid acne, rosacea Tellogen effluvium Hirsutism Ocular: Blurred vision Cataracts Glaucoma CNS: Sleep disturbance Agitation Depression Psychosis Pseudotumour cerebri Peripheral neuropathy Myopathy CVS: Weight gain, increased appetitie Hyperglycaemia HTN Hypercholesterolaemia, TG CHF Hypokalaemia GI: GORD, PUD, bowel perf Fatty liver N + V Bone: Osteoporosis Osteonecrosis Growth impairment Infections: Skin TB reactivation Opportunistic Malignancy Endocrine: Steroid withdrawal Addisonian crisis Cushingoid Menstrual irregularity Renal crisis risk if U1RNP or RNP3

Answer 875

Screen contraindications: Metabolic/CVS hx GI hx Ocular hx OP Psychosis/mental illlness Height weight BMI waist circumference BP Ophthalm - yearly DEXA scan - 2 yearly FBE UEC LFT BSL HbA1c fasting lipids Pre-immunosuppression Co-prescribe PPI Calcium, vit D Weight bearing exercise Healthy diet Quit smoking Limit ETOH PJP prophylaxis Stress dosing with surgery/severe illness

Answer 876

HSV (1>2) Mycoplasma pneumoniae EBV, CMV, parvovirus B19, adenovirus, coxsackie virus Hep B, C, HIV COVID Influenza Orf/parapox virus Drugs: NSAID, sulfonamides, anticonvulsants, other Abx, allopurinol Vaccines Systemic disease (IBD, leukaemia, lymphoma, solid organ malignancy)

Answer 877

Skin - hyperpigmentation Eyes - keratitis, uveitis, conjunctival scarring, permanent visual impairment

Answer 878

At least 6 months of continuous oral antiviral therapy (even if a clear cause has not been identified) valaciclovir 500-1000mg/day or aciclovir 400mg BD sunprotection For antiviral-resistant cases: AZA, dapsone, MMF, HCQ

Answer 879

Fever, weakness, arthralgias and joint swelling Less common: pulmonary (atypical pneumonia) Rare: renal, hepatic, haematologic

Answer 880

Typical target = at least 3 colour zones, papular. Well defined border Atypical = 2 colour zones, macular, poorly defined border

Answer 881

keratinocyte apoptosis mild spongiosis, focal vacuolar degeneration of basal keratinocytes superficial dermal oedema perivascular infiltrate of lymphocytes with exocytosis into epidermis more prominent dermal inflammatory component than SJS do not see large areas of full thickness epidermal necrosis DIF non specific - granular deposits IgM and C3 around superficial blood vessels and focally at DEJ

Answer 882

Famciclovir 1500mg orally stat (3x500mg tablets) or Valaciclovir 2g BD for 1 day

Answer 883

Same as for classic scabies: overcrowding nursing homes indigenous communities children Also: elderly immunosuppression (eg HIV, solid organ transplants, medications, lymphoma) HTLV1 decreased ability to scratch dementia

Answer 884

Sarcoptes scabiei var hominis Lives entire life within epidermis Lays 2-3 eggs/day, dies after ~1-2/12, lays 10-25 eggs in a life cycle Eggs hatch after 2-3 days Eggs --> Larvae --> Nymphs --> Adults Transmission - close physical contact with an infested person. Person to person, sexual, indirect spread via fomites Sx are due to allergic reaction to mite - delayed hypersensitivity reaction First time infestations - 2-6 weeks. Subsequent infections - pruritus within 24-48hrs

Answer 885

Ivermectin 200microg/kg orally with fatty food Permethrin 5% cream. Apply to dry skin. Under nails. Hands, genitalia. Reapply if wash hands. Leave on at least 8 hours. Benzyl benzoate 25% emulsion. Apply to dry skin. Under nails, hands, genitalia. Leave on for 24hrs and reapply to hands if washed. Higher risk skin irritation vs permethrin. sulfur (precipitated sulfur in WSP) 5-10% or crotamiton 10% Both of these applied daily for 3 days Treat post scabetic itch and secondary infection

Answer 886

5% permethrin is safe in infants >2 months (according to CDC), definitely safe >6 months Safe for use in pregnancy sulfur (precipitated sulfur in WSP) 5-10% (infants and pregnancy) crotamiton 10% option for <2 months old Both of these applied daily for 3 days Ivermectin can only be given to children >5yo and >15kg Not safe for use in pregnancy/breastfeeding

Answer 887

Ivermectin 200mg/kg PO with fatty food for 3, 5 or 7 doses depending on severity (day 1, 2, 8, 9, 15, 22, 29) (day 0, 1, 7, 8, 14, 21, 28) AND Topical scabicide (permethrin, benzyl benzoate 25% lotion +/- tea tree oil 5%) every second day for 1/52 THEN twice weekly until clinically cleared AND Topical keratolytic (SA 5-10% in sorbolene, LA 5% urea 10% in sorbolene Calmurid) daily after washing on non-scabicide days

Answer 888

PCOS CAH SAHA syndrome - seborrhoea, acne, androgenetic alopecia Cushings ACTH secreting tumour Prolactin-secreting pituitary adenoma

Answer 889

Lepromatous leprosy - Numerous lesions - Macules, papules, nodules, diffuse infiltration - Symmetric distribution - Sensation not affected Borderline lepromatous - Similar to LL, some diminished sensation BB (borderline) - Plaques, dome-shaped, punched out lesions - Asymmetry - Less well defined border - Diminished sensation Borderline tuberculoid - Can be single lesions or >5, otherwise similar to tuberculoid Tuberculoid leprosy - One or few lesions (up to 5) - Infiltrated plaques, often hypopigmented - Localised, asymmetric - Well-defined sharp borders - Absent sensation

Answer 890

- Greatest number of bacilli, least cellular immunity - Multiple, poorly defined, erythematous macules, papules, nodules, plaques - Widespread symmetric - Face, buttocks, LLs - Glove and stocking paraesthesia - Enlarged peripheral nerves SMILE EYES Saddle nose deformity Madarosis Ichthyosis Leonine facies Ear lobe infiltration Eyes - lagophthalmos, corneal/conjunctival anaesthesia Early symptoms - nasal congestion, epistaxis, pedal oedema Multiple nerve thickening Claw hand - median, ulnar Papal hand - ulnar Orchitis Foot drop "Hammer toes"

Answer 891

Grenz zone Lymphocytes, plasma cells Virchow cells - macrophages with numerous bacilli and lipid droplets in cytoplasm, foamy AFB - ZN or Fite

Answer 892

Few, well demarcated plaques Hypopigmentation Scale Anaesthesia and alopecia on plaques

Answer 893

Granulomas, no bacteria Granulomatous infiltrate may have linear pattern following a common nerve Oedematous cutaneous nerves No organisms Epithelioid/langhans giant cells

Answer 894

Sensation - pain, touch, temp Palpate nerves - Facial nerve branches, greater auricular, ulnar, radial, median, common peroneal, posterior tibial Inspect for - muscle atrophy, flexion contractures

Answer 895

Close contact + infected untreated contagious patient Risk for household contacts = 25% Wide range incubation period 2-20 years (average 5) Usually via nasal and oral droplets

Answer 896

Type 1 "upgrading" Increased cell mediated immunity Neuritis, neurological impairment - sudden onset claw hand, foot drop, facial palsy New skin lesions, increased inflammation Can occur from any leprosy but most commonly from borderline Treat: prednisolone Type 2 Erythema nodosum leprosum Vasculitis (small vessel) Occurs from lepromatous leprosy Nodular skin lesions Fever Myalgia Joint pain and swelling LNs, HSM Orchitis GN Treat: thalidomide 100-200mg daily (or CsA)

Answer 897

Histo, stains Bacilloscopy (skin slit smear) - from earlobes, forehead, chin, fingers, forearms, trunk - fold skin, firm small incision - smear - fite or ZN - red rods Historic tests - lepromin, pilocarpine, histamine PCR

Answer 898

PIMPLE J CATS Prednisolone Iodides (+bromides)/isoniazide MEK inhibitors Progestins/POP Lithium EGFR inhibitors/ antieepileptics JAKi CsA AZA Testosterone (and other anabolic steroids), tacro Sirolimus

Answer 899

Cimex lectularius

Answer 900

Black dot in endothrix - Black dot: fine scaling with patches of alopecia, which appear speckled with black dots - The “dots” are broken hair shafts secondary to endothrix infection Corkscrew hairs also more common with trichophyton

Answer 901

BSL, HbA1c TSH Fe, folate, B12, zinc Swab candida ANA, ENA, dsDNA, C3/4 Patch test

Answer 902

nose (rhinophyma) chin (gnathophyma) forehead (metophyma) ears (otophyma) eyelids (blepharophyma)

Answer 903

Lupus miliaris disseminatus faciei eyelids, forehead, cheeks and chin Untreated, it will last 2-3 years and leave scarring histo: epithelioid granulomas within the dermis, which may be caseating Treatments: Doxy OCP Isotretinoin Dapsone Pred

Answer 904

Corneal arcus

Answer 905

Foamy macrophages Fibrosis Cholesterol clefts May have occasional touton giant cell May have lymphocytes, neutrophils (if eruptive) IHC: adipophilin, oil red O

Answer 906

Avoid ETOH Diet Lipid lowering meds CO2, erbium:YAG, PDL TCA Cryosurgery Simvastatin/cholesterol cream

Answer 907

Type I, IV and V familial hyperlipoproteinaemia Hypothyroid Diabetes Nephrotic syndrome Alcohol Retinoids

Answer 908

HIV protease inhibitors (nilotinib) Retinoids Steroid CsA

Answer 909

Type II and III Fredericksons Type III = familial dysbetalipoproteinaemia (where you also get xanthoma striatum palmare) Atherosclerosis increased risk

Answer 910

50% association with hyperlipidaemia Atherosclerosis increased risk In children - associated with type IIb

Answer 911

Type II (with type II you also get intertriginous plane xanthomas in web spaces) = homozygous Frederickson type II with elevated LDL Atherosclerosis increased risk

Answer 912

Hyperlipidaemia vs normolipaemic (diffuse normolipaemic plane xanthomas) - neck, upper trunk, flexures, periorbital Monoclonal gammopathy B cell lymphoma Castleman's Xanthelasma: 50% association with hyperlipidaemia In children - associated with type IIb Intertriginous, web spaces, homozygous Frederickson Type II Xanthoma striatum palmare - familial dysbetalipoproteinaemia

Answer 913

Hyperlipidaemia Monoclonal gammopathy

Answer 914

CHILD EB GVHD Pemphigus

Answer 915

Biopsy Fasting lipids UECs, LFTs TSH Fasting BSL, HbA1c Lipase, lipoprotein electrophoresis FBE, SPEP, etc (haem disorder/malignancy)

Answer 916

TITANIC CRASH Thromboembolism Infections - URTI/nasopharyngitis/sinusitis/cough/Herpes Zoster/opportunistic Temperature increase (fever) Acne, folliculitis Nausea, abdo pain, GI perf, liver damage Increased weight CK Cholesterol/TG increase Real bad? black box IHD, clots, death Anaemia, lymphopaenia, neutropaenia Skinn cancer/lymphoma Headache

Answer 917

Hypersensitivity Pregnancy (Cat D) Breast feeding Other immunosuppression Active infections (TB, Hep B/C) Severe hepatic impairment (Child Pugh C), severely deranged ALT/AST Hb <80, lymphocytes <0.5, neuts <1 Precautions: Risk factors for thrombosis (past hx thromboembolism, obesity, immobility) Elderly >65 Severe renal impairment Malignancy CYP3A4 interactions

Answer 918

Rx by dermatologist or immunologist 12 years or older 28 days of medium to high potency TCS or CNI daily and despite that: * 2 of the EASI symptom subscores rated as severe (3) (erythema, papulation, lichenification, excoriation) * Or at least 30% of face/hands surface area Baseline DLQI Have had dx for 6 months or more Sole PBS subsidized medication No previous inadequate response To continue: Rating of 0 (none) to 1 (mild) for at least 3 of the subscores At least 75% reduction in skin area affected compared to baseline DLQI score reduced by 4 points

Answer 919

Upadacitinib 15mg daily (can increase to 30mg) Barictinib 2-4mg daily Tofacitinib 5mg BD

Answer 920

fully-human monoclonal antibody directed against the shared IL-4 receptor alpha subunit blocks signalling from both IL-4 and IL-13

Answer 921

B1, effect unknown, can cross placenta Stop 10-12 weeks before conception

Answer 922

If child <60kg = 400mg subcut, then 200mg every 2 weeks, or if <30kg then 300mg every 4 weeks

Answer 923

ISRs 1-10% Antibody development Blepharitis, dry eyes, itch eyes, conjunctivitis (10-15%), keratitis (4%) Oral HSV 2-5% URTI Nasopharyngitis Headache Gastritis Arthralgia Psoriasis Red face/residual facial erythema(consider seb derm, ACD) Eosinophilia Eosinophilic pneumonia Vasculitis LCV, ?EGPA

Answer 924

In children <4 these are suspicious places for bruising Torso Ear Neck Frenulum Auricular area Angle of the jaw Cheek Eyelid Sclera Patterned bruising Also petechial and multiple (bilateral, clustered, different planes)

Answer 925

This can be difficult to treat Avoid trauma Compression and wound care Glycaemic control Address modifiable risk factors If no diabetes - assess for other associations, yearly diabetes screen Assess for associated PVD TOP CS, CNI, retinoids, JAKi ILCS Aspirin Trental HCQ - depending on whether diabetic eye disease Doxycycline Phototherapy MMF, MTX, CsA, JAK inhibitors, apremilast, IVIG, thalidomide, colchicine, TNF inhibitors, ustekinumab laser (CO2) surgery (grafting) Monitor for SCC

Answer 926

- Diabetes - Retinopathy, nephropathy, neuropathy - Thyroid dysfunction - Ocular inflammation (retinal vasculitis) - Rheumatoid arthritis - IBD - Obesity, gastric bypass, HTN, hyperlipidaemia

Answer 927

Granulomas, necrobiosis, layered wedding cake distribution (Palisading granulomas in dermis often oriented parallel to epidermis) Inflammatory infiltrate lymphocytes, histiocytes (+ multinucleated giant cell), plasma cells, occ eos Septal panniculitis

Answer 928

Pregnancy Post inflammation eg erythroderma Malignancy - sign of Leser–Trélat - gastric or colonic adenocarcinoma, breast carcinoma, and lymphoma - malignant AN same time or shortly after

Answer 929

six histologic types of SK: acanthotic, hyperkeratotic, reticulated, irritated, clonal, and melanoacanthoma hyperkeratosis, acanthosis and papillomatosis (church spires) Horn pseudocysts sharp demarcation at the base - “string” sign

Answer 930

Acantholytic dyskeratosis at the base and sides of a cup-like epidermal invagination usually involves at least one dilated folliculosebaceous unit Cup shaped Hyperkeratotic Dyskeratoses Acantholyis

Answer 931

Epidermolytic Acanthoma Epidermal naevus Epidermolytic ichthyosis PPKDs (Unna Thost Vorner)

Answer 932

Evaluation for systemic abnormalities in conjunction with a pediatrician (CNS, MSK, eyes), hx, ex, skeletal survey Overarching statement - difficult to treat, set expectations Surgical excision (risk scars) Shave, curette (recurrence common if only epidermis removed) TCS Topical retinoid Oral retinoids Laser ablation - CO2 For ILVEN: Same as above + bleach baths (prevent secondary infection) Abx as needed Analgesia as needed + calcipotriol, simvastatin/cholesterol PDL TNF inhibitor, other biologics, apremilast, MTX

Answer 933

Tetracyclines - Oral minocycline is effective in approximately 50% of patients Retinoids Topical salicylic acid, hydroquinone, tacrolimus, antifungals (e.g. selenium sulfide), and 5-fluorouracil

Answer 934

Keratinocytic epidermal naevus - CHILD - Type 2 segmental Cowden disease - FGFR3 epidermal naevus syndrome Sebaceous naevus - Schimmelpenning syndrome - Phakomatosis pigmentokeratotica Comedo naevus syndrome Becker naevus syndrome Proteus, CLOVES

Answer 935

Chilblains/pernio - Itch and burn - Purple/red - Lasts >24 hours (may last for a week) - Associated with cold exposure/cooler weather - Response to conservative warming - Lacks pallor, cyanosis Chilblain lupus - Similar to chilblains but need evidence of lupus on histo and response to lupus treatment (+ negative cryoglobulins and cold agglutinins) Acrocyanosis - Painless - Blue - Symmetrical - Lasts longer than Raynauds - Lacks pallor - Hyperhidrosis - Ulceration, gangrene in severe cases - Primary and secondary (CTD, Buerger's, arsenic, drugs, cryoglobulinemia, MI, anorexia) - Assess presence of pulses and doppler Raynauds - Pallor, cyanosis, erythema - Cold sensitivity - Primary - ages 15-30, normal nailfold capillaroscopy - Secondary - more severe, asymmetrical, more frequent, trophic and ischaemic changes (digital ulceration, hairloss, necrosis, gangrene, autoamputation) Erythromelalgia - Redness - Increased temp - Burning pain - Triggered by heat, exercise, dependency - Improved by cold, rest, elevation - Can be bilateral or unilateral - May have cyanosis or livedo reticularis; anhidrosis/hypohidrosis; oedema; rarely ulcers

Answer 936

- pustular PsO - severe PsO - erythrodermic PsO - Asian and Middle Eastern

Answer 937

Drugs - phenytoin, nifedipine, cyclosporine Granulomatous - Crohns, sarcoid, OFG GPA Scurvy Ginigival fibromatosis - TSC, Cowdens Primary systemic amyloidosis Hormone - pregnancy, acromegaly Poor oral hygiene, periodontal disease Leukaemia Other malignancy (KS, mets)

Answer 938

FBE (anaemia, neutropaenia) Haematinics (iron, folate or vitamin B12) HIV IBD - faecal calprotectin, scopes Behcets - HLAB51 SLE - ANA, ENA, dsDNA, C3/4 Swabs Anti skin antibodies Biopsies Consider FDE, EM

Answer 939

Diet ILCS Tetracyclines HCQ Dapsone PO CS TNF inhibitors

Answer 940

Pediculosis capitis

Answer 941

>12 years old intolerant of, or has not responded to, topical aluminium chloride hexahydrate not had treatment with botulinum toxin within the immediately preceding 4 months if the patient has had treatment with botulinum toxin within the previous 12 months - the patient had treatment on no more than 2 separate occasions

Answer 942

Doxycycline 100mg BD for 14 days (ceftriaxone, azithro)

Answer 943

ABCC6 AR clumped, distorted, and calcified elastic fibers Skin: yellowish papules, flexural areas, cobblestone, plucked chicken skin, redundant folds in flexural areas - axilla and groin, loss of recoil, prominent creases on the chin Mucosa: yellow papules, most often lower lip Ocular: angioid streaks, mottling of the retinal pigment epithelium, choroidal neovascularization and haemorrhage, macular degeneration CVS: calcification of elastic fibers in the walls of medium-sized arteries, claudication, renovascular hypertension, angina and myocardial infarctions, loss of peripheral pulses, stroke GI: haemorrhage

Answer 944

Parakeratosis Mild lichenoid reaction with occasional apoptotic/necrotic basal keratinocytes Top-heavy or wedge-shaped inflammatory cell infiltrate consisting predominantly of lymphocytes distributed around blood vessels Red cell extravasation Occasional presence of lymphocytic vasculitis and T-cell clonality Negatives: Absence of CD30 positive cells, lymphoycte atypia, epidermotropism, Munro and Pautrier microabscesses

Answer 945

distorted, fragmented elastic fibers in the mid and deep reticular dermis calcium deposits on these altered elastic fibers elastin (Verhoeff–van Gieson) calcium (von Kossa)

Answer 946

ELN - AD (2/3rd de novo) ARCL1, 2 and 3 - AR Congenital or acquired Acquired - weeks to months after drug reaction, chronic urticaria, or Sweet syndrome monoclonal gammopathies penicillamine (with acquired type there is little or no extracutaneous involvement, can be emphysema and diverticula) Cutaneous: loose, sagging skin with reduced elasticity and resilience aged facial appearance, long philtrum, large ears, ptosis Extracutaneous: Emphysema umbilical and inguinal hernias gastrointestinal and genitourinary tract diverticula cardiac or arterial abnormalities - aortic root dilatation and risk of aortic root aneurysm skeletal or joint involvement (joint hyperlaxity) delayed growth and development congenital hemolytic anemia has also been reported Histo: sparse and/or fragmented elastic fibers

Answer 947

MDT renal, urology, cardiology, respiratory, genetics, ophthalmology, PT, surgery Genetic counselling, social work supports Reconstructive surgery for cosmetic concerns (may reoccur) Avoid: Positive pressure ventilation Contact with ppl with resp tract infections Smoking Sunbathing Isometric exercise Contact sports

Answer 948

Clinical: - Abrupt onset - Nodular, suppurative - Systemic/other - fevers, HSM, arthralgia, myalgia, malaise, EN, osteolytic bone lesions (clavicle, sternum) Labs: Elevated ESR, WCC Anaemia Proteinuria Associations: SAPHO CAH Anabolic steroids Treatment: Pred 0.5-1mg/kg/day for 2-4 weeks, then add low dose isotretinoin 0.1mg/kg/day, after 4 weeks of this combo, can slowly increase isotretinoin and wean pred Others: TNF, IL1, CsA, AZA TCS, ILCS

Answer 949

PCOS Normal DHEAS Elevated testosterone Elevated LH:FSH ratio >2-3 CAH 17-OH elevated Elevated DHEAS Adrenal tumour Elevated DHEAS +/- elevated testosterone Ovarian tumour Elevated testosterone

Answer 950

Hirsutism Acne Seborrhoea Roughening of skin Androgenetic alopecia/ scalp hair loss Coarsening/deepening of voice Muscular habitus Redistribution of fat more android pattern Cliteromegaly, vaginal atrophy Cessation of menses Increased libido

Answer 951

Reduced facial and body hair growth Soften skin Decreased oiliness skin Reduce muscle bulk and increase body fat Reduce sexual function, decreased libido Breast growth

Answer 952

Use of correct names and pronouns Highlighted in EMRs Non-judgmental care, open-mindedness, knowledge of TGD health concerns Prioritising patient autonomy and confidentiality (confidential time without parents for younger patients) Asking patients about gender identity, pronouns, sexual behaviours Sensitivity around discussions of contraception if sexual activity with sperm-producing partners

Answer 953

Transmasculine: - Chest binding can exacerbate acne (also pain, pruritus, miliaria, infection, contact dermatitis, scars) - More frequent liver monitoring if tetracyclines + testosterone (hepatotoxicity) - Combined OCPs can be safely used alongside testosterone therapy (although SEs like breast tenderness or menstrual spotting may contribute to gender dysphoria) - Spironolactone should NOT be used due to interference with exogenous testosterone treatment - Isotretinoin (consider surgical wound healing) Transfeminine: - Increased xerosis and irritation - Spironolactone helpful - Endocrine referral if recalcitrant

Answer 954

- Minoxidil 1.25-5mg in transmasculine Low dose 0.25-0.5mg in transfeminine to mitigate risk hypertrichosis - Spironolactone Transfeminine patients only Up to 200mg daily Improves hair density in 30%, arrests progression in 90% - Finasteride 1mg, dutasteride 0.5mg (theoretical risk of interfering with testosterone therapy) Assess severity and progress with non gender specific tools

Answer 955

Hyperandrogenism Insulin resistance Acanthosis nigricans Increased risk diabetes, cardiovascular disease

Answer 956

Trichodysplasia spinulosa BRAF (esp without MEK), CsA, acitretin) PRP (esp type 6) Follicular mucinosis Multiple myeloma

Answer 957

ACNE PHAD FA... Acne agminata - severe form granulomatous rosacea - think of with acne around eyelid, red brown papules, no comedones Chloracne - after exposure to halogenated aromatic hydrocarbons - electrical conductors/insulators, insecticides, herbicides, fungicides Naevus comedonicus Elastosis (solar), Favre Racouchot Perioral dermatitis Halogenoderma Apert syndrome Drugs Folliculotropic MF Acneiform follicular mucinosis

Answer 958

Erythematotelangiectatic rosacea Papulopustular Phymatous Ocular Granulomatous Rosacea conglobata Rosacea fulminans/ pyoderma faciale

Answer 959

Benign, usually self-limiting, asymptomatic Half of cases resolve in 2 years Recurrence possible Generalised GA may persist or clear 25% last >5 years 10% last >10 years

Answer 960

Topicals (occlusion), ILCS Top tacrolimus Cryosurgery Niacinamide Phototherapy Minocycline, doxycycline HCQ MTX, MMF, CsA, pentoxifylline, apremilast, allopurinol, isotretinoin, acitretin, statins, dapsone TNF inhibitors Laser, phototherapy, PDT, surgery JAKi under investigation

Answer 961

Generalised Localised Subcutaneous Patch Perforating Photodistributed Palmoplantar Linear Nodular Micropapular Interstitial Drug-induced

Answer 962

Hyperlipidaemia Diabetes Infections - HIV, hep B, hep C, EBV, TB Neoplasms - Hodgkins disease, NHL, MF Thyroid dysfunction Drugs - Allopurinol, amlodipine, apremilast, diclofenac, gold, levetiracetam, thalidomide, topiramate, tocilizumab, TNF inhibitors, secukinumab RA, LE IHD

Answer 963

Focal degeneration of collagen and elastic fibres Mucin deposition Perivascular and interstitial lymphohistiocytic infiltrate in upper and mid dermis Histiocyte patterns: - Interstitial or infiltrative pattern (more common) - scattered histiocytes distributed between collagen fibres - One to several palisading granulomas (less common) - with central connective tissue degeneration surrounded by histiocytes and lymphocytes Can get fibrin, C3 and IgM deposition in vessel walls and occlusion of lumina

Answer 964

LCH: Acute disseminated Chronic multifocal Chronic focal Congenital self healing reticulohistiocytosis NLCH: JXG Benign cephalic histiocytosis NXG Multicentric reticulohistiocytosis Rosai Dorfman Xanthoma disseminatum Other classification of LCH: Letterer-Siwe (involves many organs and widespread on skin) Hand-Schuller-Christian (persistent, DI, bone lesions) Eosinophilic granuloma Congenital self healing (Hashimoto Pritzker)

Answer 965

Skin bx Bone marrow bx FBE UEC LFT Skeletal survey CXR USS abdo Brain MRI BRAF testing Water deprivation for DI HRCT, lung function test Growth chart (in kids) MDM Top CS, CNI nbUVB MTX, AZA Thalidomide BRAF inhibitor if mutation PNL + vinblastine Stem cell transplant

Answer 966

Acute leukaemia Solid organ malignancy Other haem malignancy Diabetes insipidus Osteolytic bone lesions Systems: haem, pulmonary, renal, hepatosplenic, skeletal

Answer 967

Large nodular, small nodular Single or multiple Systems: bone, lung, CNS (DI), eyes 40% of children with ocular JXG have cutaneous JXG Natural hx: Childhood (infants, young) Head and neck, upper body Self resolve in 3-6 years Can get some atrophy, hyperpigmentation, anetoderma

Answer 968

Faecal - isocoproporphyrin Urine - uroporphyrin

Answer 969

Photoprotection Avoid trauma Avoid triggers Venesection: 400-500ml every 2 weeks for 3-6 months Aim ferritin 25-30 Low dose HCQ Can monitor urine - uroporphyrin

Answer 970

Blister, erosion/ulcer, scarring Most common sites: oral mucosa (especially gingival and buccal mucosa) and conjunctiva Other sites: upper aerodigestive mucosa (nose, oropharynx, larynx, esophagus), anogenital mucosa, skin COMPLICATIONS Eyes: conjunctivitis, symblepharon, ectropion, trichiasis, blindness Upper aerodigestive and esophageal involvement: odynophagia, dysphonia, esophageal stricture formation, airway compromise, loss of teeth Anogenital: phimosis, strictures of the urethra, anus, or vaginal introitus, sexual dysfunction Skin: scarring

Answer 971

Crohns/UC Haem malignancy (myeloma) Arthritis (RA), autoimmune Drugs, diabetes SLE

Answer 972

SPACED PING Sulfurs Penicillamine, penicillins ACE inhibitors Epileptics Diuretics - frusemide, spironolactone PD1 inhibitor/pembro Inhibitors NSAIDs Gliptins

Answer 973

GLUED VANT Gestational Lichen planus pemphigoides Localised (Brunsting Perry, Stump, Umbilical, Peristomal, Pretibial) Urticarial Erythrodermic, eczematous Dyshidrosiform Vesicular Vegetative Localised vulval Acral (childhood) Pemphigoid Nodularis TEN like

Answer 974

Pemphigus vulgaris - Pemphigus vegetans (Neumann severe, Hallopeau mild) - Dyshidrosiform Pemphigus foliaceous - Pemphigus erythematosus (Senear Usher) - Fogo Selvagem Paraneoplastic pemphigus IgA pemphigus - Intraepidermal neutrophilic (IEN) - Subcorneal pustular dermatosis (SPD)

Answer 975

Penicillamine Captopril and other ACEi Penicillins Cephalosporins Vancomycin Rifampicin

Answer 976

NHL CLL Castlemans Thymoma Sarcoma Waldenstrom's macroglobulinaemia

Answer 977

IgA against desmocollin 1

Answer 978

Pemphigus vulgaris, paraneoplastic SJS/TEN EM Major RIME (reactive infectious mucocutaneous eruption) MMP Behcets HSV Contact cheilitis

Answer 979

DG1,3 Plectin Epiplakin Desmoplakin 1,2 BPAG1/BP230 Envoplakin (on ELISA) Periplakin (on ELISA) A2ML1

Answer 980

aetiology unknown clonal neoplastic disorder - Some hereditary - Some harbour BRAF mutations Some cases have been linked to EBV/reaction to underlying viral infection

Answer 981

REGISCAR Red hot - fever (>38.5) Eosinophilia Glands or great big lymph nodes (lymphadenopathy) Internal organ involvement Surface area (BSA >50%), skin signs, skin biopsy Culture, chlamydia, Hep B,C, HIV etc ANA, mycoplasma - negative Atypical lymphocytes Resolution in 15 days definite (score 6 and above), probable (score 4 and 5), possible (score 2 and 3), and no DRESS (score <2)

Answer 982

Lymphadenopathy Hepatic involvement Interstitial nephritis Myocarditis Interstitial pneumonitis Myositis Thyroiditis GIT Pancreatic Cardiac and thyroid monitor 3 months, 1 year, 2 years

Answer 983

CsA IVIG MMF Cyclophosphamide, rituximab Tofa Mepolizumab Valganciclovir - if confirmed EBV, CMV, HHV6 Liver transplant

Answer 984

CDKN2A BAP1 XP MC1R POT1

Answer 985

High penetrance Uveal melanoma Cutaneous melanoma May have spitzoid pathology Malignant mesothelioma Renal cell carcinoma Cholangiocarcinoma

Answer 986

Shelterin family Telomeres Frequently spitzoid type melanomas Other cancers: Glioma CLL Thyroid Colorectal Angiosarcoma (cardiac)

Answer 987

Emergent transfer to paeds ED - skeletal survey, neuroimaging, abdominal injury screening, otoscopy, fundoscopy Phone consultation with child abuse paediatrician consider leukaemia, immune thrombocytopaenia, bleeding disorders Take good photographs with a size standard Companion diagram Interview child and adults separately Do not interview children <3 open and developmentally appropriate questions What happened here? Tell me more about that. And then what happened? documented verbatim in quotes Examine skin surface and skeleton mucosal surfaces, skinfolds, palms, soles, genitals Explanations: "It is not normal for babies this young to have any bruises. Those bruises could be the result of a bleeding issue or from some trauma that you are not aware of. Either way, your baby needs immediate further evaluation in the paediatric ED and we are going to arrange for an ambulance to take your baby there." "When children tell us that someone has caused a mark on their skin we are mandated by law to make a report to child protective services. They may contact you in the future if they choose to follow up on the report."

Answer 988

Victorian Forensic Paediatric Medical Service

Answer 989

Bleeding disorder - Von Willebrand disease, haemophilia A or B, acute lymphoblastic leukaemia Langerhans cell histiocytosis - Osteolytic bone lesions + erythema/erosions/oedema groin Mastocytosis Congenital dermal melanocytosis (Mongolian spots) Naevus of Ota (Forehead, sclera, periorbital) Naevus of Ito (shoulder and upper extremity) Ehlers-Danlos Syndrome PWS, haemangiomas

Answer 990

Methamphetamine (ice) MDMA (ecstasy) Cocaine Marijuana Opioids Alcohol withdrawal Other meds: anti-parkinsons, antibiotics

Answer 991

Olanzapine 2.5-10mg Weight gain Metabolic syndrome Lipids Sedating Akathisia CVD risk if dementia Anticholinergic (dry mouth, eyes, urinary retention, constipation) Quetiapine 50-300mg Weight gain Anticholinergic Sedating Best for if Parkinsons CVD risk if dementia Risperidone 0.5-3mg Mod weight gain Prolactin Anticholinergic CVD risk if dementia Aripirazole 5-15mg Lipids Minor weight gain CVD risk if dementia Amisulpride 200-400mg Prolactin Minor weight gain Lipids Renal excretion

Answer 992

Female Cardiovascular disease Liver disease Poor nutrition Other drugs with cardiac side effects/interactions

Answer 993

Send for microscopic exam/culture Urine toxicology Screen for anxiety, depression (GAD-7, PHQ-9) DLQI FBE, CRP (underlying infection? increased eos?) Pruritus screen Incl UECs, LFTs, thyroid, HIV ECG Weight, BMI BP Lipids, BSL

Answer 994

Peripheral lipoatrophy Central obesity, lipohypertrophy Breast and dorsocervical fat pad enlargement Adipose tissue accumulation intra-abdominal 2/12 to 2 years after ART Up to 40% NRTIs (stavudine) --> lipoatrophy PIs --> fat hypertrophy and metabolic derangement Risk factors: Age >40 CD4 <200 Longer duration ART Associations: T2DM, IR, HTN, CVD, TG, osteoporosis/avascular nercrosis Treatment: Goals - cosmetic concerns, systemic associations, metabolic derangements Fillers (PLLA, calcium hydroxylapatite) Autologous fat transfer Lifestyle mod Liposuction Screen and treat metabolic (statins, metformin)

Answer 995

Exclude: Malignancy Ill fitting dentures Oral candidiasis Xerostomia Contact dermatitis Fe, zinc, B12, folate T2DM Hypothyroidism Menopause Treatment: Low dose benzos Antidepressants Gabapentin TOP antifungals TOP capsaicin ~0.025% TOP anaesthetics CBT

Answer 996

Stage 0 - Melanoma in situ Stage I (ie if < 1mm or <2 no ulceration) - A = T1a <0.8mm or T1b = <8 ulceration or 0.8-1 +/- ulceration - B = T2a = 1-2 mm no ulceration Stage II - >1mm with ulceration or >2 +/- ulceration Stage III - Regional nodal and/or intra lymphatic metastases Stage IV - Distant metastases

Answer 997

AD LMX1B Usually thumb Nail absent or hypoplastic Triangle lunula Absent or hypoplastic patella Radial head dysplasia Iliac crest exostoses ("horns") Nephropathy in 40% Renal insufficiency in 8%

Answer 998

Clinical variants: hyperpigmented, nonpigmented, giant, subcutaneous, multiple grouped, disseminated multiple, ankle type, pedunculated, hyperkeratotic Histological variants: cellular aneurysmal epithelioid atypical lipidised ankle type palisading

Answer 999

AICTD eg lupus, atopic dermatitis, immunosuppression (HIV)

Answer 1000

AD LEMD3 Dermatofibrosis lenticularis disseminata Connective tissue naevi Osteosarcoma, osteopoikilosis Elastomas Nasolacrimal obstruction Deafness Diabetes Ocular - nystagmus, strabismus Restricted aortic valve Fibrosis (muscle fibrosis)

Answer 1001

somatic activating mutations in AKT1 vascular malformations with variable capillary, venous and lymphatic components asymmetric, disproportionate overgrowth --> severe deformity and disabling orthopaedic consequences cerebriform connective tissue nevi of the palms and soles epidermal nevi lipomatous overgrowth regional absence of fat Bullous pulmonary degeneration specific neoplasms (e.g. ovarian cystadenoma, parotid monomorphic adenoma) facial phenotype with ptosis and anteverted nares DVT PROTEUS Progressive overgrowth Palmoplantar connective tissue naevi R - hunched shape of back (kyphoscoliosis), disproportionate overgrowth, atrophy upper arm muscles and neck Respiratory - lung cysts Orthopaedic and soft tissue overgrowth, disproportionate and asymmetric Thromboembolism Epidermal naevi and lipomas U - V - vascular and lymphatic malformations Spleen and thymus overgrowth

Answer 1002

Dystrophic Metastatic Mixed Idiopathic Iatrogenic

Answer 1003

TAPING Trauma Autoimmune Panniculitis Infection Neoplasm Genetics Trauma - heel sticks, injection sites, keloids/surgical scars AICTD DM - esp juvenile, anti-NXP2, finger tip ulceration, longer disease duration SSc Less commonly lupus, morphoea Panniculitis (esp lobular) - pancreatic panniculitis, lupus profundus, and subcutaneous fat necrosis of the newborn Infections Onchocerca volvulus Other worms or larvae Neoplasms Pilomatricoma, BCC, pilar cysts, epidermal cyst Genetic disorders PXE, EDS, PCT, Rothmund Thomson

Answer 1004

Renal disease - hyperparathyroidism Milk-alkali syndrome Hypervitaminosis D Sarcoidosis Familial tumoral calcinosis

Answer 1005

Idiopathic Calcified Nodules of the Scrotum Tumoral calcinosis (sporadic) Subepidermal Calcified Nodule Milia-like calcinosis

Answer 1006

Extravasation of IV calcium Organ transplant, esp liver Gadolinium (nephrogenic systemic fibrosis) Calcium paste for EEGs After calcium alginate dressings

Answer 1007

Albright hereditary osteodystrophy Fibrodysplasia ossificans progressiva Progressive osseous heteroplasia Plate-like osteoma cutis

Answer 1008

Megalencephaly Capillary malformation Polymicrogyria PIK3CA (PROS) Asymmetric overgrowth Widespread reticulated CM Progressive (hemi)megalencephaly Syndactyly, polydactyly Neuro: hypotonia, seizures, mild to severe intellectual disability

Answer 1009

PIK3CA PWS/CM VM (combined CVM or CLVM) Progressive overgrowth of affected extremity Leg length discrepancy - orthopaedic care Lymphoedema Some may also have affected - anogenital area, bladder, pelvis, retroperitoneum, and gastrointestinal tract that may bleed and result in anemia Check D-dimer and fibrinogen level - clot risk Prophylactic anticoagulation esp if other risk factors (surgery, pregnancy)

Answer 1010

Diffuse capillary malformation with overgrowth Widespread reticulated CM Sharp midline demarcation anterior trunk Proportional soft tissue and/or bony hypertrophy Overgrowth not progressive and does not correspond to area of CM No VM or LM Normocephalic No associated neurological abnormality GNA11

Answer 1011

Congenital Lipomatous Overgrowth (which is asymmetric, progressive, infiltrative; and typically truncal) Vascular anomalies Epidermal nevi Scoliosis/Skeletal abnormalities - include broad hands and feet Spinal/paraspinal arteriovenous lesions Seizures PIK3CA

Answer 1012

CM of the lower lip LM of tongue or neck Asymmetry and Partial Overgrowth of the face and extremities PIK3CA

Answer 1013

Vascular malformations - error in development of blood vessels/formation of vascular structures Vascular tumours - proliferation of normal cells or structures

Answer 1014

Very common, occurs in 30-80% of neonates remnants of the foetal circulation rather than a mosaic condition due to a somatic mutation characteristic locations on the forehead/glabella in a V-shape (“angel kiss”), eyelids, philtrum, occiput, nape (“stork bite”), and lumbosacral area more prominent with crying or vigorous activity tends to fade spontaneously between 1 and 3 years of age (extrafacial and glabellar can be more persistent)

Answer 1015

Facial port wine stain (forehead high risk) Brain/cerebral and ocular leptomeningeal vascular malformations, causing: - seizures, neurodevelopmental delay, stroke - glaucoma, optic atrophy, blindness Bone/soft tissue overgrowth Endocrine - hypothyroid, growth hormone deficiency

Answer 1016

1. 25-50% 2. >50%

Answer 1017

MDT Urgent referral to neurology - monitor for seizures, MRIB Urgent referral to ophthalm - eye exam (can have gluacoma from birth) Prophylactic aspirin (may help reduce stroke/seizures)

Answer 1018

80% get at least 50% better 20% excellent response 20% poor response

Answer 1019

Skin - pain, bleeding, ulceration Venous - phleboliths, DVT/PE Reduced mobility Loss of function - digits, limb 2nd to overgrowth Emaciation/wasting of unaffected tissues

Answer 1020

dark purple to red–purple, broad reticulated vascular pattern intermingled with telangiectasias and occasionally prominent veins one or more limbs and the corresponding quadrant(s) of the trunk persists upon warming atrophy, ulceration, scarring up to 50% of patients have hypoplasia (girth > length) of the affected limb(s) often lightens after 1 year may also have neurological defects, ocular (glaucoma) and skeletal (syndactyly) Ddx: Neonatal lupus CM associated syndrome - DCMO, MCAP Cutis marmorata Adams Oliver syndrome - malformation limbs, nail dystrophy, syndactyly, brachydactyly, oligodactyly, cardiac defects, pulmonary hypertension, developmental delay

Answer 1021

Angioma serpiginosum CLUSTERS/"lagoons" of tiny punctate telangiectasias in serpiginous pattern NON-BLANCHABLE (vascular laser) Unilateral naevoid telangiectasia Segmental configuration Reticular configuration BLANCHABLE (sclerotherapy)

Answer 1022

AD Genes: ENG (HHT1), ACVRL1 (HHT2 visceral AVMs and mucocutaneous telangiectasias telangiectasias face, lips, tongue, palms and fingers, including periungual areas and the nail bed First sign epistaxis - usually begins in childhood ~age 12 HHT1 higher risk cerebral AVMs HHT2 higher risk liver AVMs Screening: Transthoracic contrast echocardiography (bubble study) - pulmonary AVMs Brain MRI - cerebral AVMs Genetic testing If >35 years old - annual haemoglobin and haematocrit testing - to detect bleeding from GI AVMs If signs/symptoms of heart failure or hepatobiliary disease - USS - Detect hepatic AVMs

Answer 1023

Gene: ATM AR Ataxia initially - in toddlers Telangiectasias age 4-6 - conjunctivae, face, and ears Immunoglobulin deficiencies (e.g. IgA, IgG) and defective cell-mediated immunity Sinopulmonary infections lymphoma and leukemia risk Elevated α-fetoprotein Cariers also have increased risk breast and haem malignancy

Answer 1024

Somatic activating mutations in TEK (50%) and PIK3CA (25%) Blue Soft, compressible Fills with dependency best imaged using T2-weighted MRI D-dimer, fibrinogen

Answer 1025

GVMs: painful when palpated partially (rather than fully) compressible Does not swell with exercise or dependency Hyperkeratotic with a cobblestone-like appearance Much more common to have fam hx (2/3rds as opposed to 1% with classic VMs)

Answer 1026

Congenital lymphedema e.g. Milroy Usually before age 1 Sporadic or familial Lymphedema praecox Around puberty e.g. Meige disease Lymphedema tarda Usually after age 35

Answer 1027

THIN VISOR Thrombosis (post DVT) HS Infections - filiariasis, recurrent cellulitis Neoplasia Venous disease Immobility Surgery Obesity Radiotherapy

Answer 1028

Arterial bleeding Thrombosis/sagittal sinus thrombosis Secondary wound infection Cerebral infection Encephalocele Heterotopic brain tissue Meningocele

Answer 1029

Sign of ectopic neural tissue or membranous aplasia cutis

Answer 1030

Dermoid cysts - skin coloured, firm - sinus ostium Dermal sinuses Cephalocoeles - encephalocele blue, soft, compressible, may transluminate Heterotopic brain tissue (nasal gliomas) - red, firm, non-compressible Nasolacrimal duct cyst Lymphatic malformation Other (non developmental): Epidermoid cyst Pilomatricoma Infantile haemangioma Neurofibroma Malignant - neuroblastoma, osteosarcoma, fibrosarcoma, rhabdomyosarcoma

Answer 1031

Infant Noted birth/early Fam hx NTDs Hx of meningitis Neuro signs or symptoms Location midline or over suture lines Pulsate, transilluminate, fluctuate in size with crying/straining Overlying CM Hair collar sign

Answer 1032

Midline: Hypertrichosis/ "faun tail" Lipomas Infantile haemangiomas Telangiectasias (may represent IH-MAG) Vascular malformations Dimple (above gluteal cleft, >2.5cm from anal verge) Dermal sinuses Acrochordons Aplasia cutis congenita Tail and pseudotail Connective tissue naevus Hypo/depigmentation CMN Subcutaneous masses which may represent lipomas, meningoceles

Answer 1033

Localised or widespread areas of skin are absent or scarred at birth Sharply marginated Oval/round Glistening pink membranous plaque Alopecia Hair collar (specify no ulcer or brain tissue or bone on view)

Answer 1034

Obesity menstrual irregularities diabetes mellitus pituitary and thyroid disorders

Answer 1035

Sunprotection Monitor for SCC (esp linear) 2% cholesterol, 2% simvastatin cream 5-FU Calcipotriol treatments Oral acitretin or isotretinoin Cryotherapy CO2 laser ablation Dermabrasion

Answer 1036

LEOPARD Carney's complex Peutz-Jeghers Laugier-hunziker Cronkhite-Canada Cowdens

Answer 1037

1. Normal 2. Dilated capillary nail folds with preserved density 3. Dilated capillary nail folds with reduced density 4. Haemorrhage, neovascularisation, avascular area (drop out), giant capillaries

Answer 1038

Scleroderma-dermatomyositis like pattern Telangiectasia Ragged cuticle Periungual erythema Vessel drop out Avascular areas Capillary haemorrhages Giant/enlarged capillaries Dilated capillaries Bushy capillaries Dotted vessels Hairpin capillaries Crisscross capillaries Brownish pigmentation

Answer 1039

Cutaneous xanthomas Mucous membrane xanthomas Diabetes insipidus Corneal and conjunctival lesions Symmetric, flexural, intertriginous Rarer associations: Monoclonal gammopathies Thyroid disorders Rx: Radiotherapy for airways Cyclosporine, cyclophosphamide Surgery, cryotherapy

Answer 1040

Intraepidermal lymphocytes (without overt atypia) Perivascular lymphocytic infiltrate (can be quite mild) Red cell extravasation Haemosiderin in dermis (Perl's stain) Endothelial cell swelling May be some spongiosis, or some lichenoid change Relevant negatives: no vasculitis, no apoptotic keratinocytes ddx: MF, stasis dermatitis, lichenoid or spongiotic process

Answer 1041

Hyperkeratosis Papillomatosis Hypergranulosis Columns of parakeratosis, especially over dermal papillae (church spires) (More orthokeratosis between) Dilated capillaries in dermal papillae (with haemorrhage) Inward pointing of the rete reidges Perivascular lymphocytes Koilocytes = keratinocytes with small shrunken nucleus and surrounding halo/vacuolisation Large pink inclusions Stains: not usually required, can do PCR for HPV type

Answer 1042

Flat wart architecture Bubbly bluish cytoplasm in keratinocytes Large cells with perinuclear halo May have cytologic atypia Can transform to SCC HPV 5, 8

Answer 1043

Storiform or cartwheel pattern of thin spindled fibroblasts Very cellular Extends into subcut fat Honeycomb pattern trapping of fat/ infiltration of fat Mild to moderate cytological atypia Very few mitoses No foamy cells or multinucleated giant cells (which you can get in DF) CD34+ Factor 13A negative S100 negative

Answer 1044

Epidermal hyperplasia, flattened/tabled rete ridges (or a basaloid proliferation simulating a BCC) Hyperpigmentation at basal layer Poorly circumscribed proliferation of boomerang-shaped spindled fibroblasts Multinucleated giant cells, foamy histiocytes or sometimes Touton giant cells Haemosiderin Keloidal collagen/ collagen trapping often at periphery

Answer 1045

Encapsulated - with cellular areas (Antoni A tissue) and oedematous myxoid areas (Antoni B tissue) - frothy, foamy Spindle cells in Antoni A tissue lined up in parallel rows/pallisading Verocay bodies (pink areas without nuclei separating the spindle cells) Sclerotic hyalinisation around vessel walls Can get mast cells Can get haemorrhage Positive staining for S100 and MBP Mucinous stroma

Answer 1046

Not necessarily really encapsulated (like scwannoma), but well circumscribed/sharply demarcated dermal tumour/nodule - sometimes might be a capsule Palisading of spindle cells Crack like clefting artefact around them Don't really have Verocay bodies like in Schwannoma Positive staining for S100 MBP Neurofilament stain (more in this than in Schwannoma)

Answer 1047

AN Acrochordons Hyperhidrosis Striae distensae Intertrigo, cutaneous candidiasis Lymphoedema Lipodermatosclerosis Stasis dermatitis Leg ulcer Plantar hyperkeratosis Frictional hyperpigmentation

Answer 1048

telogen effluvium lanugo-like hair Xerosis generalized pruritus carotenoderma Hyperpigmentation Acrocyanosis Perniosis Livedo reticularis May have signs of specific nutritional deficiencies (e.g. pellagra, scurvy), poor wound healing, oedema, seborrheic dermatitis Petechiae interdigital intertrigo Paronychia particularly calluses or scars on the knuckles or dorsal surface of the hand (Russell's sign) due to repeated self-induced vomiting over prolonged periods of time Enlarged salivary glands (Bulimia) Erosions of tooth enamel (Bulimia)

Answer 1049

Hartnup Infection - HIV Alcohol Carcinoid IBD, insufficiency (dietary, Whipples, gastric bypass) Medications - isoniazid, phenytoin, 5-FU, AZA, valproic acid, carbamazepine

Answer 1050

Cerebral haemorrhage Femoral sheath haemorrhage Vasomotor instability Weakness, malaise Depression Anaemia Retinal haemorrhage Haemarthrosis

Answer 1051

Vitamin B3 Niacin deficiency dermatitis, diarrhea & dementia Photodistributed erythema that becomes scarlet or hyperpigmented, with scale-crust; surface has shellac-like appearance “Casal necklace” – well-demarcated band around the neck Painful fissures of the palms & soles Cheilitis & glossitis (atrophic, red) Peri-anal & oral inflammation & erosions Vaginitis with ulceration Systemic: Peripheral neuropathy with dysesthesias, including burning Oesophagitis Lassitude Anorexia Dizziness Abdominal pain Irritability, disorientation Late – irritability, anxiety, apathy, diarrhea, nausea, dementia

Answer 1052

Alopecia, sparse/brittle hair Follicular hyperkeratosis Angular cheilitis Glossitis Poor wound healing Petechiae, purpura, ecchymoses Nails - koilonychia, soft/thin/slow growth Corkscrew hairs Perifollicular haemorrhage Bleeding gums Gingival hypertrophy Xerosis Seborrheoic dermatitis Photodistributed shellac like scale Perioral dermatitis Conjunctivitis, blepharitis Erythema, erosions, scale-crust

Answer 1053

ATP7A XLR defective copper absorption low copper levels in the blood, liver and hair appear normal and develop normally until 2 to 3 months of age failure to thrive, lethargy, hypothermia, and hypotonia Seizures, developmental delay, anaemia, bony deformities Pudgy cheeks, a cupid's bow of the upper lip, and horizontal eyebrows Alopecia Hair light in colour, sparse, fragile and kinky Pili torti segmental shaft narrowing (monilethrix) brush-like swellings of the hair shaft (trichorrhexis nodosa) diffuse cutaneous pigmentary dilution due to decreased activity of tyrosinase

Answer 1054

Protein C, S Homocysteine Factor V Leiden Antithrombin III Antiphospholipid antibodies Factor 8 Prothrombin

Answer 1055

Schamberg - most common - Yellow–brown patches with an oval to irregular outline - Pinpoint petechiae within patches – likened to “cayenne pepper” - favours lower legs (can involve rest of limbs and trunk) Purpura annularis telangiectodes of Majocchi - 1–3 cm annular plaques - slowly expand - form concentric rings - Punctate telangiectasias and petechiae within the border - Trunk, proximal lower extremities Pigmented purpuric lichenoid dermatitis of Gougerot and Blum - Admixture of two types of lesions: (1) Schamberg-like; (2) Purpuric red–brown lichenoid papules Eczematid-like purpura of Doucas and Kapetanakis - Scaly petechial or purpuric macules, papules and patches - Lower extremities - Pruritic Lichen aureus - Solitary patch - Colour varies from golden to rust to purple–brown - Often over a perforator vein/ varicose vein Linear pigmented purpura - Linear array, usually unilateral - Often a single extremity

Answer 1056

Classic Erosive Hypertrophic Lichen planopilaris

Answer 1057

PN Atopic dermatitis Scabetic nodules Insect bite reaction Prurigo nodule variant of BP Lichen planus Lichen planus pemphigoides Hypertrophic lichen planus Dermatitis herpetiformis Perforating dermatosis - reactive perforating collagenosis or acquired perforating dermatosis Eruptive KAs

Answer 1058

dense lichenoid infiltrate in the upper and mid dermis predominance of plasma cells vascular proliferation/ectasia extravasated red cells haemosiderin “lozenge-shaped” keratinocytes

Answer 1059

Acanthosis, hypergranulosis Large and atypical nuclei Coarse nuclear chromatin and nuclear hyperchomasia disordered maturation of keratinocytes (especially with usual type VIN ie HPV related; less disordered with differentiated type ie LS/LP related) Very basaloid with 'usual type'

Answer 1060

Aureus Epidermidis Saprophyticus Haemolyticus Lugdenensis

Answer 1061

Phthirus pubis Permethrin 1% shampoo Permethrin 5% Dimethicone 4% Malathion 0.5% Ivermectin

Answer 1062

AD (or sporadic) No fragility

Answer 1063

Macroconidia coming from hyphae

Answer 1064

Beaded hair Normal at birth, then replaced by short fragile brittle hair in first few months Perifollicular erythema and hyperkeratosis AD KRT86, 81 No cure Gentle hair care, avoid excessive combing and washing TOP minoxidil Oral acitretin, griseofulvin, minoxidil, corticosteroids

Answer 1065

CAFES Comedonal Acral haemorrhagic Flexural Erosive, vesiculobullous Segmental 1 and 2 Other: nipple hyperkeratosis, isolated palms and nail changes

Answer 1066

Xanthoma disseminatum Lobomycosis Keloidal morphoea or scleroderma DFSP Carcinoma en cuirasse

Answer 1067

Adalimumab Infliximab Etanercept ethinyl estradiol/levonorgestrel (OCP) Lithium Vemurafenib Rituximab Cyclosporine Tocilizumab

Answer 1068

Hypertrophy and folding of the scalp Primary (idiopathic) or Secondary Associations with primary: neurologic and/or ophthalmologic abnormalities (ID, seizures, cataracts, optic atrophy) Associations with secondary: MAGNITT Myxoedema Acromegaly Graves Noonan's Insulin resistance Turner's TSC Others: Klinefelter, Hyper IgE syndrome, vemurafenib, paraneoplastic Ddx: - pachydermoperiostosis cerebriform - congenital melanocytic nevus of the scalp - primary systemic amyloidosis - Leukaemia cutis - benign hamartomas or tumors (plexiform neurofibroma, connective tissue naevi) - dissecting cellulitis of the scalp - Goeminne syndrome - Beare–Stevenson cutis gyrata syndrome

Answer 1069

Juvenile Hyaline Fibromatosis and Infantile Systemic Hyalinosis hyaline accumulation in the dermis AR CMG2 Infantile systemic = more severe (visceral involvement, recurrent infections, diarrhoea) Papulonodular lesions ears, scalp, neck, hands, periorificial areas, perianal gingival hypertrophy flexion contractures of large joints - severe physical limitations

Answer 1070

Primary (favours young females) Secondary - in same site as a previous specific skin lesion OR associated with an underlying disease HIV APLS Autoimmune thyroiditis, SLE, Sjogrens Syphilis Penacillamine JXG, molluscum, varicella, hep B vaccine, GA, sarcoid, lymphocytoma cutis, insect bites, mastocytosis, prurigo nodules Pilomatricoma, DF, DFSP, IH, MF, nodular amyloid Familial (AD and AR forms) Anetoderma of prematurity

Answer 1071

Striae: atrophicae, gravidarum, distensae, caerulia, rubrae, nigrae, albae puberty, pregnancy, rapid growth, weight gain or loss, obesity, hypercortisolism Treatments: Top tretinoin Top glycolic acid 20% Top L ascorbic acid (vit C) 10% Striae albae - 308 nm excimer laser Striae rubrae - PDL Ddx: linear focal elastosis

Answer 1072

Erythema migrans Borrelia lymphocytoma - weeks to months after tick bite - erythematous nodules - earlobe, nipples, scrotum, nose or arms Acrodermatitis chronica atrophicans

Answer 1073

Acroangiodermatitis and underlying AVM - think of this especially if macular pigmentation over toes

Answer 1074

Can be: Sporadic Inherited AD Part of “keratosis pilaris atrophicans” group (incl keratosis follicularis spinulosa decalvans and ulerythema ophryogenes) Or a/w syndromes Loeys–Dietz Rombo Nicolau–Balus (syringomas and milia) Treatment: Dermabrasion, laser therapy (e.g. CO2, 585 nm pulsed dye), and fillers (e.g. hyaluronic acid, autologous fat)

Answer 1075

XLR keratotic follicular papules on the malar area and progresses to involve the eyebrows, scalp and extremities, with associated scarring alopecia

Answer 1076

atrophoderma vermiculatum ulerythema ophryogenes erythromelanosis folllicularis facei et coli keratosis pilaris rubra faciei

Answer 1077

Paroxysmal extreme pain disorder Complex regional pain syndrome Fabry's disease Peripheral neuropathy Raynaud's

Answer 1078

Classically Pseudomonas aeruginosa Other bacteria: other Pseudomonas spp. (P. cepacia, P. maltophilia), Serratia marcescens, Aeromonas hydrophila, Klebsiella pneumoniae, Vibrio vulnificus, Moraxella spp., Morganella morganii, Escherichia coli, and Staphylococcus aureus Fungal: Candida albicans, Mucor spp., Aspergillus fumigatus, Fusarium spp. and Neoscytalidium dimidiatum

Answer 1079

arterial or coronary catheterization acute thrombolytic therapy anticoagulation eg warfarin, typically after 1-2 months

Answer 1080

fever, weight loss, myalgias, altered mental status, and sudden onset arterial hypertension Transient ischemic attacks, strokes, renal failure, gastrointestinal ulcerations, and hemorrhagic pancreatitis

Answer 1081

Surgical bypass or endovascular stent grafting to eliminate the source of embolism Aspirin and other antiplatelet agents Discontinuation of triggering anticoagulation OR initiation of anticoagulation in patients with severe renal damage Statin Systemic corticosteroids Iloprost Hyperbaric oxygen

Answer 1082

Primary (idiopathic) or secondary Secondary associated with: chronic venous hypertension and varicosities hypercoagulable states (APLS, facot V leiden mutation, protein C/S or antithrombin 3 def, hyperhomocystinaemia) Treatment: antiplatelet, anticoagulant, and fibrinolytic therapies Pentoxifylline Anabolic agents such as danazol and stanozolol Prostanoids (e.g. alprostadil [PGE-1]), rivaroxaban, and IVIg HCQ Ddx: With atrophie blanche - Chronic venous insifficiency - APLS - Malignant atrophic papulosis (Degos disease) - Ulcers secondary to hydroxyurea - PAN

Answer 1083

T & B Cell: iTV - Infections - Tattoo - Vaccines T Cell: iACD - Insect bite/scabies - Actinic reticuloid/CAD - Contact - lymphomatoid - Drug - lymphomatoid B Cell: MVB - Varicella - Molluscum - Borrelia

Answer 1084

Acral pseudolymphomatous angiokeratoma of children Type of pseudolymphoma Favours extremities of children ages 2-6 Unilateral grouping of small, red to violet, “angiomatous” papules

Answer 1085

Moniliform blepharosis

Answer 1086

Ulcerans Marinum Fortuitum* Abscessus* Kansasii Chelonae* Haemophilum Avium complex *rapidly growing

Answer 1087

Inverse Vesicular Pustular Purpuric

Answer 1088

malnutrition mycobacterial infections (tuberculosis and leprosy) underlying malignancies (e.g. hepatocellular carcinoma, gastric carcinoma, multiple myeloma) familial

Answer 1089

Small plaque parapsoriasis: Parakeratosis and spongiosis Large plaque parapsoriasis: Same, but also interface lymphocytic infiltrate with a variable degree of lichenoid features

Answer 1090

Chlorambucil + pred Cyclophosphamide Radiation CO2 laser Plasmapheresis

Answer 1091

Destructive arthritis Haem or solid organ malignancy (25-30%) - lung, breast, ovarian, cervical Hyperlipidaemia Positive tuberculin test Systemic vasculitis Autoimmune disease (15%) Can get fever, malaise ?diabetes, cardiac 50% have mucosal membrane involvement Coral beads tongue Leonine facies

Answer 1092

NSAIDs, corticosteroids, MTX, cyclophosphamide, TNF inhibitors

Answer 1093

Neuromas - traumatic - palisaded encapsulated - multiple, non-encapsulated Schwannomas - Common, solitary - Ancient - Cellular Neurofibromas - Plexiform - Diffuse - Superficial - Deep - Pigmented Malignant peripheral nerve sheath tumour Meningocele Nasal glioma Neuroblastoma Neuroendocrine: Granular cell tumour Merkel cell carcinoma

Answer 1094

MEN2B Cowdens (Bannayan Riley Ruvalcaba)

Answer 1095

NF1, Noonan syndrome, Leopard syndrome

Answer 1096

Epidermal hyperplasia, sometimes pseudoepitheliomatous hyperplasia Granular cells in dermis - large cells with granular cytoplasm and small, centrally located nuclei Larger eosinophilic intracytoplasmic granules = pustulo-ovoid bodies (of Milan) Perineural involvement common Mitoses are rare (look for these and atypia to see if rare malignant variant) Stain: S100, SOX10 CD68 (lysosomes) Granules PAS positive side note: commonly on the tongue, so see if it is squamous epithelium or skeletal muscle underneath

Answer 1097

Poorly circumscribed (not necessarily though) Hypercellular Epithelioid cells Spindle cells Mitoses More cytoplasm, more infiltrating and less mucin than ordinary neurothekeoma Multilobular/nested/micronodular Cells - oval to round nuclei With pale, fine, delicate chromatin Stains: NEGATIVE for S100 Positive NSE stain, S100A6, PGP9.5, NKI-C3 SMA may be positive in 50%, desmin negative ddx: Spitz Clues: looks nested like could almost be melanocytic, but no epidermal involvement

Answer 1098

Also can be called nerve sheath myxoma Pale myxoid sharply demarcated or encapsulated dermal or subcutaneous nodule divided into lobules or fascicles by fibrous septae Spindle cell nuclei, can be epithelioid, may be pleomorphic Mucinous stroma S100 positive

Answer 1099

Clue: might look like Pyogenic Granuloma Purple smudgy colonies (amphophilic areas) - containing numerous bacilli - will stain with Warthin-Starry or Giemsa Surrounding granulation tissue with neutrophils; vascularity; macrophages; lymphocytes There may be fibrosis which may stain positive for Factor XIIIa Negative for HHV-8 and CD31

Answer 1100

Tufted angioma Congenital melanocytic naevus Smooth muscle hamartoma Hairy naevus/fawn tail naevus Fibrous hamartoma of infancy