General Medical cards Dan Flashcards
Which icthyosiform syndromes have PPK?
‘Never Kid THe lipid’ Nethertons KID syndrome THiodystrophy with icthyosis - IBIDS/PIBIDS Neutral Lipid Storage Disease
List the uncommon causes of multiple facial papules
‘MMR ABCDE’ AAAA BB CC
Muir-Torre (sebaceous adenoma, carcinoma, sebaceoma, keratoacanthoma. NB seb carcinoma usually away from face in Muir-Torre)
Milia en plaque (PXE, DLE, LP)
Rosai Dorfman disease
Angiofibromas (TSC, MEN1, Birt-Hogg-Dube)
Acne agminata
AD pilar cysts
Apocrine hidrocystoma (Schöpf–Schulz–Passarge syndrome)
Birt-hogg-Dube (Fibrofolliculomas, acrochordons, Trichodiscomas) or familial trichodiscoma
Brooke-Spiegler (trichoepithelioma, cylindroma, spiradenoma) or AD form of any of these
Cowdens syndrome (trichilemmomas, cobblestone facial papules)
Colloid milium (can look like syringomas, trichoeps or hydrocystomas)
Down syndrome (syringomas, can also be sporadic)
Eruptive vellous hair cyst (Pachyonychia congenita or familial trait)
Causes of erythema nodosum?
No cause found in 60%
MY Bi-Lateral SORE SHINS
Mycoplasma Or pneumococcal pneumonia Or psittacosis
Yersinia infection (enterocolitis)
Behcets, Bartonella (cat scratch dx)
Leprosy
Strep infection
OCP (also pregnancy) + other drugs SHOPS in Gold Mine
Rickettsia
EBV and HepB
STDs; HIV, syphilis, chlamydia, gonorrhoea
Histoplasmosis, blastomycsis, coccidioidomycosis
IBD (UC>Crohns)
NHL
Sjogren’s, Sarcoid and TB (granulomatous diseases)
What are the ARA diagnostic criteria for SLE?
4 out of these 11 needed to potentially label the patient having SLE;
MD SOAP BRAIN;
Malar rash
Discoid rash
Serositis such as pleuritis or pericarditis
Oral ulcers
Arthritis (usually oligo or polyarticular)
Photosensitivity
Blood dyscrazias: hemolytic anaemia, leukopenia, lymphopenia & thrombocytopenia
Renal involvement with nephrotic picture
ANA (95% of patients)
Immunological abnormalities such as Anti-Sm, Anti-dsDNA, Anti-phospholipid, false positive syphilis serology
Neurological/Psych: mainly seizures and psychosis
List the Annular (figurate) erythemas and their associations
Erythema (chronicum) migrans – tick bite/Lyme disease
Erythema gyratum repens –malignancy
Erythema annulare centrifugum – infection, lupus, malignancy
Erythema marginatum (rheumaticum) – acute rheumatic fever
List your Dermatology Sieve
SIGN VIP MEN (O) Squamous Infective Granulomatous Neoplastic Vascular Immune Physical/traumatic Metabolic Endocrine Nutritional (O)other – drug side effects
What grouping is Streptococcus Pyogenes?
What are the major post strep complications?
Strep pyogenes = Lancefield group A, Beta haemolytic strep
Complications? STREP SICKS
Derm:
Sweets disease
Toxic shock syndrome
Reccurent toxin-mediated perineal erythema
Erythema nodosum, Erythema marginatum
Psoriasis, guttate, PPP(pustular bacterid), Pit lichenoides
Scarlet fever
Infection: Impetigo, cellulitis, erysipelas, ecthyma, perianal strep etc
CSVV (vasculitis) and PAN
Kawasaki disease
Scleredema (type 1)
Non derm:
Glomerulonephritis
Rheumatic fever
PANDAS
Other strep infection – sinusitis, pneumonia, septic arthritis, osteomyelitis, meningitis, vaginitis
What is drug induced SLE?
Who gets it?
What are the features?
Acute lupus-like syndrome with fever, arthralgia, myalgia and pleuritis
ANA often positive
Affects people with HLA-DR4 and slow acetylators preferentially.
F:M = 4:1
No nephritis or CNS features
Uncommon in black people, older age of onset than sporadic SLE
Unusual to have cutaneous lupus specific lesions but may have non-specific skin features. Except if due to TNF inhibitor then can have any kind of cutaneous LE
Usually associated with anti-histone Abs except if due to TNF inhibitors – ANA + anti-dsDNA;
or Minocycline – p-ANCA
Especially remember MyHyPIE
Minocycline
Hydralazine - high risk
Procainamide - highest risk
Isoniazid
Etanercept/TNFalpha blockers
what are the Toxic Erythemas?
STAK Scarlet fever Toxic Shock syndrome Acute GVHD Kawasakis disease
What genoderms are associated with cafe au lait macules?
Cheer leader with CALMs spins the BATANS Bloom's syndrome Albright (McCune-Albright) syndrome (coast of Maine) Tuberous sclerosis Ataxia telangiectasia NF1 Silver-Russell (Russell-Silver) syndrome Also; Idiopathic NF1-like syndrome (Legius syndrome) NF2 Watson syndrome (Allelic to NF1) - CALMs, pulmonary valve stenosis, short stature Noonans syndrome LEOPARD syndrome (cafe noir, CALM, lentigines) Carney complex (cafe noir, lentigines) Fanconis anaemia Gorlin’s Cowdens (sometimes) MEN1 (sometimes) Mafuccis Gaucher Chediak-Higashi Hunter syndrome Multiple mucosal neuroma syndrome Epidermal naevus syndromes
What is Graham Little syndrome?
Subtype of LPP
LP, KP, scarring alopecia, non-scarring hair loss;
4 features not necessarily at same time;
1. Progressive cicatricial alopecia
2. Classical LP lesions of skin or mucosa
3. Non-scarring loss of pubic + axillary hair
4. KP with long spines in plaques on trunk, limbs or face
What is SCORTEN?
How is it scored?
Assess SJS/TEN pts for SCORTEN after 24hrs in hospital using highest scores and again on day 3. 1 point each; ABC PUSH; Age over 40 Bicarbonate below 20 mmol/L Cancer Percent BSA >10% initially and reaches >30% Urea over 10 mmol/L Sugar - BGL over 14 mmol/L Heart rate over 120
Predicted mortality; 0-1 - 3% 2 - 12% 3 - 36% 4 - 60% 5-7 - >90%
List your DD for sporotrichoid spread (nodular lymphangitis)
Infections Most common; Atypical mycobacteria esp M. marinum (also kansasii, chelonaei, Gordonae) Deep fungal infection esp sporotrichosis (S schenckii), also other dimorphic fungi e.g Blastomycosis Rare; NB; Nocardiosis esp N. brasiliensis (pyogenic) Bacteria - staph, strep Very rare in developed world; Like TO TAG Cats And Cows Leishmaniasis Tuberculosis Opportunistic fungi e.g Fusarium spp, Alternaria spp. Tularaemia Anthrax Glanders (B. mallei) or melioidosis (pseudomallei) Cats scratch disease Acanthamoeba spp Cowpox NB Orf and Milkers nodules can simulate this pattern if multiple lesions on an extremity Non-infective causes and mimics; Lymphoma Langerhans cell histiocytosis In transit metastases e.g melanoma Perineural spread (mimic) e.g leprosy, skin cancers
Which drugs are high risk for SJS/TEN?
SATAN drugs - also cause DRESS Sulphur drugs esp sulphonamides Allopurinol Tetracyclines Anticonvulsants NSAIDs esp COX2 and oxicams
what is Rowell’s syndrome?
Lupus erythematous + EM-like lesions
with RF+ve + Speckled ANA or Anti-Ro or La
What is metabolic syndrome?
Need 3 out of 5 of; HOT Bacon Butty HDL low or treated Obese - elevated waist circumference >102m/88f cm Triglycerides high or treated BP high or treated (>130 sys /85 diast) BSL>5.5
Causes 2-3x risk DM or CVD, 1.6x mortality risk
What are causes of septal panniculitis?
ASPEN migration + vasculitis and infection;
Alpha1 antitrypsin def (lob>septal)
Scleroderma/ morphoea profunda
PAN
Erythema Nodosum + EN migrans (+ eosinophilic panniculitis)
NLD, GA
Superficial or migratory Thrombophlebitis
CSVV and infective causes
- as well as CSVV, vasculitis is seen in PAN and thrommbophlebitis types
What are the drugs that cause erythema nodosum?
SHOPS IN Gold Mine Sulphur drugs - sulphonamides, sulphonylureas Halides - Bromides, Iodides OCP Penicillin Salicylates IsotretinoIN NSAIDs Gold Minocycline
What are the diagnostic criteria for Kawasaki disease?
what is management?
CRASH and burn (Kawasaki motorbike)
Conjunctivitis
Rash - polymorphous/macpap/scarletiniform
Adenopathy - cervical
Strawberry tongue + lips red and fissured
Hand swelling/erythema/desquamation
Burn = high fever; over 38 degrees for over 5 days
need the fever and 4 of the other 5 features to make the diagnosis
Mx;
blds; raised WCC, high ESR, high plts
echo
refer - paeds, cardiology
High dose aspirin + IVIg;
80-100mg/kg/day divided over four times per day for two weeks then reduce for next 6-8 wks
IVIG 2g/kg IV over 10-12hrs as single dose; can rpt
What are the categories of acanthosis nigricans?
What are some common causes?
Melissa Manahan SAD HO Mixed Malignant Syndromic Acral (tripe palms) Drug (steroids, hormones) Hereditary Obesity
causes - PODGE PCOS or HAIR-AN Obesity Diabetes Gastric cancer adneocarcinoma and other malignancy Endocrine – Other endo cause; Cushing’s, Acromegally also; congenital generalized lipodystrophy
What is PHACES syndrome?
Who gets it?
How do you procede?
Association of a large segmental facial infantile haemangioma with one or more malformations.
90% are female;
PHACES
Posterior fossa defects (Dandy-Walker malformation most common)
Haemangiomas
Arterial anomalies (esp carotid artery)
Cardiac defects (coarctation of aorta most common)
Eye abnormalities
Sternal clefts or pits, supraumbilical raphe (=ventral developmental defects)
Upper face lesions linked with brain,cerebrovascular and eye abnormalities
Lower face lesions linked with ventral developmental defects
Scan head if concerns
What are the features of Sturge-Weber syndrome?
STURGE Leper (leptomeningeal vascular malformation)
Sporadic (not inherited)
Tram track calcification (on skull X-ray)
Unilateral portwine stain (sometimes central forehead)
Retardation (learning difficulties)
Glaucoma/angioma of choroid
Epilepsy
Other features;
Hypertrophy of gingivae and other tissues e.g. bone - can occur with any PWS capillary malformation not only in SWS
As can eye enlargement, myopia
What are the features of lumbosacral infantile hameangioma? What is management?
(L)LUMBAAR – Lumbosacral haemangioma, often ulcerated Lipoma or other skin lesions Urogenital anomalies Myelopathy (dysraphism) Bony deformities Anorectal malformations Arterial anomalies Renal anomalies investigate even if neurologically normal – USS under 4 months of age, MRI if older
What is Mafucci’s syndrome?
Presents in infancy
Cutaneous venous malformations + enchondromas
Deformed hands and feet
High risk of malignancy - enchondromas can transform into chondrosarcomas.
Also angiosarcomas, lymphangiosarcomas, fibrosarcomas, osteosarcomas
Also CALMs
F/u long term
What is Blue rubber bleb syndrome?
Sporadic or AD
Usually presents in adulthood
Multiple venous malformations of skin, gut, spinal cord and viscera - skin lesions are painful
No glomus cells
What are the side effects of EGFR inhibitors?What other targeted agents have same side effects?
Eg. Cetux, pmab, erlotinib, gefitinib
‘EGFR makes(MEK) a fast car’ (rhymes) - if you have a fast car you get ‘MPH PRIDE’
Mucositis
Photosensitivity
Hair changes eg hypertrichosis, hirsuitism, trichomegally of eyelashes + Alopecia (androgenetic or rarely scarring)
Papulopustular eruption
Regulatory changes in hair
Itching
Dryness
Easy breaking nails (brittle) + paronychia + onycholysis + pyogenic granulomas
NB; MEK inhibitors have same side effects eg. Seletanib, trametinib
What is HAIR-AN syndrome?
Due to insulin resistance in women Subtype of PCOS. Triad of; Hyperandrogenism HA Insulin resistance IR Acanthosis nigricans AN
What is the Arthus reaction?
Rare type of local immune complex (type 3) hypersensitivity vasculits due to injection of an antigen, usually esp diphtheria or tetanus toxoid in a sensitized individual.
Local pain, swelling, redness and sometimes purpura or necrosis in 4-10 hrs then settles.
What is Carney complex?
AD lentiginosis syndrome Mutation in PRKAR1A gene Cutaneous lentigines + atrial myxomas + endocrine tumours and overactivity 2 subsets: NAME and LAMB Think Name the Lamb PATT Naevi Atrial Myxoma Myxomas of skin Ephelides Lentigines Atrial myxoma Mucocutaneous myxomas Blue naevi Pituitary adenoma Adrenal Cushings Thyroid tumours Testicular tumours
What conditons are knuckle pads asociated with?
Idiot Kids Traumatized By Fist Knuckle Pads
Idiopathic in kids
Trauma
Bart-Pumphrey syndrome
Fibromatoses
Keratin 9 Epidermolytic palmoplantar keratoderma - Vorner’s
Papillon-Lefevre syndrome
What is POEMS syndrome?
variant of myeloma Polyneuropathy - sensory and motor Organomegally - Liver, spleen LNs Endocrinopathy - mainly DM M protein - monoclonal gammopathy Skin changes - hyperpigmentation, hypertrichosis, scleroderma, Terry's nails treat with chemo and autologous SCT
What is Achenbach’s syndrome?
Spontaneous onset of painful haematomas/bruises on fingers in middle aged females
Unknown cause but lesions often triggered by minor trauma
Usually resolves
Which syndromes are associated with multiple lipomas?
Lipoma syndromes; Dercum's Family Makes Bull Shit Other syndromes; 1,1,P10, He-man's Garden Dercum’s disease (adiposis dolorosa) – painful lipomas Familial multiple lipomatosis Benign symmetrical lipomatosis NF1 MEN 1 PTEN syndromes; Bannayan-Riley-Ruvalcaba Proteus Cowden's lumbosacral haemangioma Gardner's syndrome
what are the causes of painful dermal tumours?
Blue ANGEL or BLEND AN EGG Blue rubber bleb Angiolipoma Neuroma Glomus tumour Eccrine spiradenoma Leiomyoma
Blue rubber bleb Leiomyoma Eccrine spiradenoma Neuroma - Morton's, traumatic Dermatofibroma (sometimes) Angiolipoma Neurilemmoma Endometrioma Glomus tumour Granular cell tumour also Tufted angioma in 30% of cases
What are the key features of Pellagra?
4 D's Dermatitis Diarrhoea Dementia Death
What are the cutaneous features of antiphospholipid syndrome?
U LAD Ulceration Livedo reticularis Atrophie blanche, Acrocyanosis Degos-like lesions
What is the follicular occlusion tetrad?
If you find one, always look for the others;
- Hidradenitis suppuritiva
- acne conglobata
- dissecting cellulitis of scalp
- Pilonidal sinus
Diagnostic criteria for DRESS?
STAMPER - 7 diagnostic, 6 possible;
Symptoms persist >2 wks after stopping drug
Temp >38 degrees
ALT >100; hepatitis or other visceral inflammation
Maculopapular rash >3wks after starting drug
Palpable LNs
Eosinophilia or high WCC or raised atypical lymphocytes >5%
Re activation of HHV6
What are the top drug causes of AGEP?
Mostly antibiotics Also antifungals, diltiazem, paracetamol + quinolones, HCQ Do My Pits Pus Today? Diltiazem Macrolides Penicillins, beta lactams Plaquenil Terbinafine and azole antifungals
What are the causes of erythroderma?
Common derm; Eczema + variants (seb derm, stasis, contact) 40% Psoriasis 25% PRP CTCL (MF or Sezary) 15% Pemph foliaceous or other immunbullous Idiopathic 10% Rare derm causes; HOSTING Lovely ladies Double Ds Hailey-hailey Ofuji's papuloerythroderma Sarcoidosis Toxic Shock Syndrome Icthyoses Norwegian scabies GvHD Lupus Lichen planus Dermatophyte Dermatomyositis Non derm causes; Drugs - 10% - esp CASA - Carbamazepine/phenytoin - Allopurinol - Sulphasalazine - Ampicillin/penicillinsSystemic lymphoma/leukaemia Hypereosinophilic syndrome HIV seroconversion
What are the complications of skin emergencies?
THE INET + Metabolic
Thermoregulation + Thrombosis
Haemodynamic – ARF, CHF/high output cardiac failure, odema
Ectropion + other eye complications
Infection – skin, pneumonia etc
Nutrition, Nails & Nodes (low albumin marker of poor protein status)
Enteropathy (iron, B12, folate, protein and fat malabsorption and deficiency)
Telogen effluvium
Metabolic - electrolyte imbalance
What is APECED syndrome?
Autoimmune PolyEndocrinopathy Candidiasis Ectodermal Dystrophy syndrome Type 1 autoimmune polyendocrine syndrome Addisons Hypoparathyroidism Immune deficiency Skin features - CHAV nail; Candidiasis - chronic mucocutaneous Hyperpigmentation of Addisons Alopecia areata Vitiligo - in most cases Nail dystrophy
What are the diagnostic criteria for antiphospholipid syndrome?
Need at least 1 clinical and 1 lab feature
Clinical;
- One or more episodes of arterial, venous or small vessel thrombosis
- Pregnancy complications
One or more unexplained foetal loss at 10 wks or later (normal foetus)
One or more prem delivery of normal neonate before 34 wks
3 or more unexplained consecutive miscarriages before 10 wks
Laboratory (remember LAB);
- Positive Lupus anticoagulant Ab test on ≥2 occasions ≥12 wks apart
- IgM or IgG Anticardiolipin Abs at mod-high levels on ≥2 occasions ≥12 wks apart
- Positive Beta2-glycoprotein 1 Abs test on ≥2 occasions ≥12 wks apart
NB thrombocytopenia is no longer in the diagnostic criteria
Which plants are common causes of phytophotodermatitis?
ARM and Leg - rash seen on extremeties
Apiaceae (Umbelliferae)
- Parsnip
- Celery
- carrots
- Parsley
- Cow parsley/hogweed
- Giant hogweed
Rutaceae
- Bergamot lime (Citrus bergamia) also causes berloque dermatitis
- Citron (Citrus medica)
- Pomelo (Citrus maxima)
- Ruta spp ('Rue')
Moraceae
- Fig
Leguminosa
- Beans
NB; Phytophoto is a type of phototoxic rcn mainly due to plant furocoumarins, not a photoallergy
compositae [asteraceae] cause ACD but not photoACD or phototoxicity
- Ragweed and parthenium cause airborne bush dermatitis
However it is theorised that a reaction occurs between plant material in this species and human DNA which renders the patient photosensitive accounting for hight rates of compositae allergy in pts with CAD
Define: Koebner phenomenon Pseudo koebner Reverse Koebner Isotopic response Pathergy
Kebner (isomorphic resposne) = skin condition occuring at site of a scar (healed)
Pseudo Koebner phenomenon = infection such as warts or molluscum at sites of trauma
Reverse Koebner phenomenon = trauma to a site of dermatosis causes resolution
Isotopic response = dermatosis occurring at the site of a previously healed, unrelated dermatosis
Pathergy is more appropriate terms in Behcets, Sweets and PG if the wounds are new
What is Rosai-Dorfman disease?
‘Sinus Histiocytosis with massive Lymphadenopathy’
rare type of non-Langerhan’s cell, Non-dendritic cell histiocytosis
affects age 10-30, M>F, all races
>600 reported cases
- Massive, painless cervical lymphadenopathy
- Fever
- Mild anemia and neutrophilia, high ESR
- 40% have extranodal involvement with skin being most common site (bone, CNS, genitourinary, GIT, heart, thyroid)
Skin involved in 10%; can be only extranodal site involved + rare skin-limited form
Multiple red-brown or xanthomatous macules, papules, nodules or plaques of eyelids and malar regions
Often indolent, self-limiting
Many lesions are asymptomatic and heal spontaneously Rare fatal cases
Poor prognosis if; disseminated LN Dx or involvement of liver, kidneys or lungs
What are the types of Porokeratosis?
7 types;
Mibelli
Giant
DSP/DSAP (if only on sun-exposed areas)
Linear
Porokeratosis palmaris, plantaris et disseminata (PPPD)
Punctate porokeratosis (may be variant of PPPD)
Porokeratotic Eccrine Ostial and Dermal Duct Naevus (PEODDN)
What is BLAISE?
Blaschko Linear Acquired Inflammatory Skin Eruption includes lichen striatus in children and adult blaschkitis
What is Salzberger-Garbe disese?
AKA Oid-Oid disease or
Exudative discoid and lichenoid chronic dermatosis
widespread disocid eczema - lesions go through exudative and lichenified (not lichenoid) stages
mainly adult jewish men, cause unknown
penis and scrotum invovlement pathognomonic
Very itchy
can be eosinophilia
can be gynaecomastia
treatment resistant - try pred or AZA
Chronic - months-years
What are the DSM IV diagnostic criteria for trichotillomania?
- Recurrent pulling of ones own hair causing hair loss
- Increasing sense of tension when resisting and immediately before hair pulling
- Pleasure, relief or gratification after pulling out hair4. Not part of another mental disorder
- Causes problems or distress with social life or work or other areas of functioning
Can support diagnosis by creating a ‘hair growth window’ – weekly shave a small area of involved scalp and see normal confluent growth at end of week
What are the associations of geographic tongue
Psoriasis Eczema Scrotal/fissured tongue Downs Diabetes Reiter's syndrome hormonal disturbance Anaemia Lithium EGFR inhibitors
What are the associations of scrotal tongue?
Melkersson-Rosenthal syndrome Down syndrome Less often; Cowdens syndrome Acromegally (with macroglossia) Pachyonychia congenita
