Dermatology Flashcards

Question

Acral Lentiginous melanoma

Answer 1

5-10% of all melanomas: - Most common subtype in darker-pigmented individuals - 60-72% in African Americans - 29-46% in Asians Median age of onset being 65 years old - Most common site is the sole - Not all palmar or plantar melanomas are ALMs (minority are SSMs or NMs) Appearance: - Variegation in color and irregular borders - May be mistaken for plantar wart or hematoma More advanced lesion upon diagnosis associated with poorer outcomes ** ALM is not thought to be associated with sun exposure

Answer 2

Variant of ALM: - Generally arises from the nail matrix - Most commonly on the great toe or thumb - A widening, dark, or irregularly pigmented longitudinal nail streak (melanonychia striata) +/- Nail dystrophy Hutchinson sign= - Pigmentation of the proximal nail fold - Poor prognosis, associated with advanced subungual melanoma

Answer 3

A: Asymmetry – one half is not identical to the other half B: Border – irregular, notched, scalloped, ill-defined C: Color – Varying shades from one area to the next D: Diameter – > 6 mm or pencil eraser E: Evolving * Other reasons for changes in mole: - Inflammation - Folliculitis - Trauma - Hormonal influence - Natural evolution

Answer 4

Cytologic atypia: - Cellular enlargement - Nuclear enlargement - Nuclear pleomorphism - Hyperchromasia of nuclei - Nucleolar variability - Mitoses Architectural disorder: - Asymmetry - Poor circumscription - Variation in size of nests of melanocytes in the lower epidermis and dermis - Lack of maturation of nests with descent into the dermis - Pagetoid spread

Answer 5

AKA Neurotropic melanoma Indurated papule, plaque, nodule Pigmentation absent in at least 40% of cases (i.e. amelanotic) Typical locations: - Head and neck (53.2%) - Extremities (26.2%) - Trunk (20.6%) Mean age 63 years old Diagnosis is delayed due to non-specific clinical features Histopathologically, it may simulate fibrosis/scar or a neural neoplasm leading to misdiagnosis and inappropriate treatment

Answer 6

Pink/skin colored raised bump- harder to ID as nevi

Answer 7

Most important= Breslow thickness - Measured from the granular cell layer to the deepest penetration of the neoplasm - Survival decreases with increasing tumor thickness Melanoma ulceration - Marker for aggressive nature of lesion - Nodular lesions - Up stage equivalent to one T level Mitosis - New prognostic factor as of 2009 - Proliferation of the primary melanoma - Threshold considered significant is greater than 1 mitosis per mm2 - Adversely influences survival - Melanoma measuring < 1mm in thickness Anatomic site - Worse prognosis: Head and neck, Trunk, Hand and feet; Five-year survival 84% - Better prognosis: Arms and legs; Five-year survival 93% Age - Increasing age--> decreasing survival - Patients older than 50 years of age have a 84% five-year survival vs. 90% for younger patients Gender - Female patients have a better survival - Female five-year survival 90% - Men five-year survival 83%

Answer 8

Status of regional lymph nodes most powerful prognostic factor for survival - Presence of regional lymph nodes portends a worse prognosis - Number of lymph nodes involved is the most significant risk factor The second most important risk factor is tumor burden - Micrometastatic disease (SLN Bx) vs. macrometastatic disease (clinically palpable disease) Worse prognosis: - Ulceration - Satellites - Both clinical and microscopic metastasis around a primary melanoma - In-transit metastasis: Intralymphatic metastases between the primary tumor and its nodal basin

Answer 9

Site of metastasis – most important factor - Visceral metastases poorer prognosis than non-visceral (skin, subcutaneous tissue, distant lymph nodes) sites - Number of metastases - Amenable to surgical resection

Answer 10

Pathophys: - Multifactorial disorder with genetic predisposition - New immune cell recognised: TH17 cell--> increased IL 17 causing increased keratinocyte proliferation--> chemokine release--> skin defense Risk factors: - Strep pharyngitis - Stress - Skin trauma (scratches): Koebner phenomenon Features: - Nail pitting (v. difficult to treat)- increased risk of psoriatic arthritis - Morning stiffness

Answer 11

Skin fold regions (inverse of expected region) | Does not have silver plaques

Answer 12

Defined by erythema and desquamation involving more than 90% of skin surface Differential: - Skin eruption - Infection - Allergic reaction

Answer 13

May be systemically ill - Need to be followed/assesed for: hypocalcemia, hypotension, fever Care must be given with systemic steroids as taper--> pustular flare

Answer 14

Local: - Corticosteroids - Vit D analogues - Tar Phototherapy: - PUVA - Narrowband UVB Systemic: - Retinoids (push cell differentiation) - Methotrexate - Cyclosporine - Mycophenolate mofetil - TNF-alpha inhibitors for psoriatic arthritis

Answer 15

3/4 major features: 1. Prutitus 2. Typical morphology and distribution 3. Chronically relapsing 4. Personal or family history of atopy Usually not before 3 months of age - Relies on patient sratching it In infancy: - face= common site - sites where they can rub against bed, reach easily - May see failure to thrive Childhood/adulthood - Flexural surfaces= common sites - Chronic involvement can lead to lichenification (skin thickens) Features: - White dermatographism (not red wheal, but white wheal) Theory behind pathophys: - Immunologic abnormalities (humoral immue, t-cell mediated immune response) - Structure of skin abnormal (BAD skin) - Defect in humoral immune response: high levels of serum IgE, binding to langerhaans cells - Antigen-induced activation of IgE-bearing cells (mast cells, macrophages) - Decrease in cyclic AMP--> hyperreactive mast cells, Langerhans' cells Exacerbating factors: - Anxiety, stress - Climatic factors: temps, humidity - Irritants: detergents, solvents, wool/rough material - Food, contact, inhaled antigens

Answer 16

Pruritis precedes visible eruption - C-fibers transmit itch first Scratching--> increased release of histamine from mast cells--> pruritis

Answer 17

Differential diagnosis: - Psoraisis, scabies, eczema - Wiskott-Aldrich (eczema, thrombocytopenia, immunodeficiency) - Hyper-IgE (cold staph abscesses- not painful/hot, eczema-like rashes, lung infections) - Cutaneous T-cell lymphoma

Answer 18

Earlier onset=typical Absent pruritis Limited distribution on face, groin - Greasy scaly macules and patches on the central part of the face (nose, mouth, and eyebrows). - Scalp is a common site of involvement, which the patients complain of bad “dandruff.” The condition is seen in neonates (e.g. cradle cap), which resolves when the maternal hormones are washed out. - The condition is usually not seen in prepubescent age. In adults, the condition is more common in patients with neurologic diseases such as Parkinson’s disease. Cause: belief that condition is caused by a commensal fungus, namely, Malesezzia furur. Treatment: antifungal creams and shampoos are often recommended such as ketoconazole cream and shampoo. Other treatments include zinc based soaps and shampoos, immunomodulators such as pimecrolimus, and low potency steroid.

Answer 19

Papules, burrows, nodules Distribution: - Infants= generalized - Adults: finger webs, wrists, exillae, groin Pruritis in patient and contacts Mite debris on microscopic examination

Answer 20

Disseminated Herpes Simplex Virus (HSV) Painful umbilicated pustular vesicles Fever, malaise, lymphadenopathy Significant morbidity and mortality (risk of systemic herpes infection) Seen in patients with atopic dermatitis or other chronic skin conditions: - dissemination of the cutaneous herpes simplex virus into the eczematous skin may lead to the eruption of clusters of umbilicated pustular vesicles known as eczema herpeticum or Kaposi's varicelliform eruption

Answer 21

Pustular vesicles with straw-colored discharge | - Due to staph aureus infection

Answer 22

- Topical steroids or immune modulators: tacrolimus, pimecrolimus - Antihistamines - Wet wraps - Coal tar in ointment base, anthralin - Antibiotics (topical, oral) - Leukotriene antagonist Flares: - PUVA, UVB - Systemic steroids - Immunomodulators (cyclosporine, tacrolimus, azathioprine)

Answer 23

Pruritic disorder of skin, mucous membranes Appearance: violaceous polygonal papulaes, plaques on penis - Hypertrophic or erosive Oral mucosa with typical white reticulated plaque - Associated with HCV Erosive lichen planus= increased risk of squamous cell carcinoma in oral/mucosal lichen planus - v. painful, can cause scarring/disfigurement

Answer 24

Variant of lichen planus Seen in children - non-pruritic

Answer 25

Chronic folliculitis--> scarring ressembling keloids in darker-skinned individuals

Answer 26

Cause: Staph - Furuncle= single follicle - Carbuncle= multiple follicles Treatment with I&D and antibiotics - Chronic abscesses due to carriage

Answer 27

Inflammatory non-infectious process Multi-focal and symmetric involving 2+ locations - Treatment is difficult, scarring results

Answer 28

Firm, mobile nodules | Treatment: I&D

Answer 29

More common in kids, caused by Group A strep erythrogenic exotoxin - 24-48 hours after sore throat - Sandpaper-like papules - Pastia lines, desquamation of palms and soles Diagnosis: strep culture, ASO Treatment: antibiotics

Answer 30

Group A strep (except in post-partum/newborns= Group B strep) - Infection of superficial dermal lymphatics from lacrations - Erythematous, brawny plaque on face, legs Treatment: oral antibiotics

Answer 31

Staph or strep Port of entry: tinea pedis in toes Usually unilateral, can be bullous Treatment: IV antibiotics

Answer 32

Chronic venous stasis--> extravasated RBCs - Erythema, yellow-brown plaques with lichenification - Often bilateral Treatment: compression, topical steroids

Answer 33

Borrelia burgdorgeri= Lyme disease - 3-32 days after bite as papule--> annular - 25-50% develop multiple lesions - Fades without treatment Treamtent (for Lyme): doxycycline

Answer 34

Varicella= Chicken pox Herpes Zoster

Answer 35

Cytomegalovirus In adults: similar to EBV infection In neonates: TORCH syndrome - Blueberry muffin baby Can also co-infect with HSV Treatment: gangciclovir

Answer 36

Roseola - Self-limited, seen in babies Pityriasis Rosea: - Viral exanthem - 20% have mild flu-like symptoms - begins with 2-10 cm oval, scaling patch for 1 week--> followed by smaller oval patches (cigarette paper scale "christmas tree") - Treatment: topical steroids for itch (rash recedes on its own * Mimicker= secondary syphillis (6-8 weeks post-chancre)

Answer 37

Caused by Malasseziafurfur - Well-demarcated hypo/hyperpigmented patches on skin Treat: topical antifungals

Answer 38

T. rubrum, T. Metagrophytes, M. Canis, T. tonsurans - Annular lesions with peripheral scale Diagnosis: KOH stain Treatment: topical antifungals Mimicker: seborrheic dermatitis - More common in adults than tinea - Pityrosporum ovale - Greasy scale over erythematous plaques (sebaceous areas) - More common in patients with neurologic disease - Treatment: topical antifungals

Answer 39

Dermatophyte invades and ruptures hair follicle Mimicker: nummular dermatitis - Commonly found on extremities - round, erythematous plaques with scale - Spongiotic dermatitis - Treat with topical steroids

Answer 40

Subcutaneous mycoses: chromoblastomycosis - Caused by Fonasacea pedrosoi - Verrucous plaques, nodules - Diagnosis: sclerotic bodies on H&E Treatment: oral antifungals

Answer 41

M. fortuitum, M. chelonei, M. Abscessus - Infections linked to surgical procedures and nail salon baths Treatment: debridement, combination antibiotics

Answer 42

Immune-compromised, debilitated patients HUGE mite load - Hyperkeratotic lesions BOTH topical or oral therapy needed

Answer 43

1. Impetigo: staph, s. pyogenes 2. Bullous impetigo: staph exfoliative toxin 3. Staph "scalded skin" syndrome: no bacteria in skin samples Mimicker: 1. Pemphigus foliceous: auto-antibodies to desmoglein 1 (cell adhesion complex) 2. Toxic epidermal necrolysis: necrosis of epidermis (deeper splitting)

Answer 44

Epstein Barr virus Can cause morbiliform eruption 7-10 days after amoxicillin/ampicillin treatment

Answer 45

Most common malignant neoplasm in humans Most common form of skin cancer, and now most common form of cancer in United States Rarely metastasizes, but can be locally destructive Associated with long term chronic UV exposure 75% of non-melanoma skin cancers 99% in Caucasians Majority in patients 40 years and older but not uncommon in 20-40 year olds 85% on head, neck, and trunk

Answer 46

``` Pigmentary phenotype-Fitzpatrick I-II Ultraviolet light exposure- intense episodic Radiation exposure Pre-existing skin lesions - nevus sebaceous Genetic Syndromes - Basal cell nevus syndrome - Xeroderma pigmentosa ```

Answer 47

Nodular - Smooth translucent, pearly papule, overlying telangiectasia +/- ulceration, pigmentation Micronodular - More plaque like with less defined borders Superficial - Erythematous or atrophic macule with raised peripheral borders Infiltrating/Sclerosing - Scar like plaque with ill defined borders

Answer 48

Range of clinical findings include: - Numerous BCCs (photodistribution), - palmar pitting, - hypertelorism, - ondontogenic cysts, - bifid ribs, - brachymetacarpalism, - calcification of falx cerebri, - partial agenesis of corpus callosum, - ovarian fibromas, - medulloblastoma Genetics: - Transmission sporadic or autosomal dominant - Chromosomal defect 9q p22

Answer 49

Excision: 1. Conventional (3-4mm margins)- vertical sections 2. Mohs micrographic surgery- sectioned vertically and horizontally: Destruction- ED&C, Cryotherapy Immunomodulators- - Topical imiquimod (Aldara™) Radiation - elderly, poor operative candidates, or palliation

Answer 50

- Recurrent BCC - Ill defined borders - Cosmetically important area/Tissue sparing - Size > 2.0cm - Increased risk of recurrence: Location on nose, ears; infiltrating or micronodular histology Principles: - Excision of tumor as complete block - Horizontal embedding technique - Peripheral and deep margins embedded on same horizontal plane - 100% margin evaluation - Intraoperative histologic evaluation and mapping of residual tumor - Additional tissue layers taken selectively until negative margins - Allows for immediate reconstruction

Answer 51

- 20% of non melanoma skin cancers - Uncommon but not rare in non Caucasians - Male:Female ratio= 3:1 - Increased incidence with increasing age >40 - Occurs on areas of chronic sun exposure: Head, neck, dorsal hands

Answer 52

``` Pigmentary phenotype- Fitzpatrick I, II Ultraviolet Radiation- chronic recreational, therapeutic, PUVA Chemical carcinogens Chronic ulceration or scar (Marjolins ulcer) HPV Immunosuppression (transplant- heart 65x increased risk) Genodermatoses (XP, EDV) ```

Answer 53

AK is precursor to SCC - Clinically erythematous scaling macule or papule on sun damaged skin - Can regress, remain stable, or progress to SCC Malignant conversion rate ~ 1/200 or 1/1000 per year - 10% risk of SCC in 10 years with 10 AKs Treatment : liquid nitrogen, topical 5FU, imiquimod (Aldara™)

Answer 54

SCC in situ (Bowen’s) - erythematous scaling macule SCC- invasive - Erythematous nodule or eroded plaque SCC/KA type (keratoacanthoma) - Rapidly growing nodule with central keratin plug Verrucous carcinoma Erythoplasia of Queyrat- - moist red plaque on genitalia

Answer 55

High risk for recurrence, metastasis ``` Perineural involvement (must check patient for palsies, pain, etc) - spreads along cleavage plane between nerve and sheath (seen in 2-14% of all SCC, 1% of BCC) ``` Invasion into bone Recurrence

Answer 56

``` Skin Cancer (SCC) - Peak effect at 300nm (UVB) in mice Photoaging - Elastosis - Irregular pigmentation - Telangiectasia - Peak effect at 340nm (UVA) ``` Mechanisms: 1. DNA Damage - Pyrimidine dimer formation - Activation of oncogenes or inactivation of tumor suppression genes - Damage to two alleles leads to neoplasia 2. Immunologic Suppression - Decreased immune surveillance by down regulation of Langerhans cells - Altered cytokine production

Answer 57

Candidate gene for BCNS localized to chromosome 9q22.3 - PTC - putative tumor suppressor gene - Two hit hypothesis: 1. BCNS associated with inherited mutations in one allele 2. UVR produces additional defective allele leading to neoplasia The "patched" gene= - Tumor suppressor gene that encodes a transmembrane protein in ‘Hedgehog’ signaling pathway - regulates cellular proliferation and organ development during embryogenesis - Implicated in the development of sporadic BCC and other tumors: lung, prostate, pancreatic, gastric, hepatocellular, neural Treatment: 1. Cyclopamine (inhibits HH pathway) - Steroid alkaloid and teratogen - Foundin Rocky Mtn wildflower= Veratrum californicum 2. Vismodegib: FDA-approved BCC tx

Answer 58

Tumor suppressor gene on chromosome 17p13.1 - Mutations found in 50% of human cancers including skin cancer Transcription factor for genes controlling apoptosis - Loss of suppressor functions can lead to neoplastic proliferation Mutations in skin cancers are pyrimidine dimers c/w UV induction - Mutations in 60% of AKs, 90% of SCC, 50% of BCC - P53 mutations prevent apoptosis of UV damaged cells - Additional UVB can lead to clonal expansion, carcinogenesis

Answer 59

Rare AR genodermatoses Defective DNA endonuclease - Inability to perform normal DNA excision repair Clinical features: - Severe actinic damage, early onset cutaneous neoplasia - Cataracts, photophobia, keratitis - May be associated with neurologic impairment (De Sanctis-Cachione syndrome)

Answer 60

UVA: - Penetrates into epidermis/dermis - Not filtered by glass - Leads to dermal collagen damage - Seen throughout day, all year UVB: - Absorbed in epidermis - Erythema of sunburn - Seen in late spring, summer, autumn, midday Both: - Cause photo aging - Skin cancer (more UVB)

Answer 61

Minimal erythemal dose - Smallest dose of UV radiation that can induce sunburn - Not necessarily correlated with skin type Ex: Burn after 1 minute of sun exposure - SPF 30= 30 minutes to get burned from sun exposure

Answer 62

``` Follicular accentuation Lichenification Post-inflammatory pigmentary alteration Excoriation Picker's nodules/ Prurigo nodularis Ulcers/erosions ```

Answer 63

Primary: skin diseases: - Xerosis (dry skin, eczema craquele, senescence) - Scabies - Urticaria (comes and goes rapidly) - Dermatitis herpetiformis - Urticarial stage of bullous pemphigoid - Contact dermatitis Iatrogenic: - Medication-induced pruritis Secondary: Systemic disease - Chronic renal disease - Liver disease (cholestasis) - Iron deficiency - Polycythemia vera - Thyroid disease - Parasitic infestation - Lymphoma - Diabetes

Answer 64

No effective specific antipruritic drugs: - Topical agents (corticosteroid, phenol, menthol, opioid) - Oral antihistamines - Systemic corticosteroids - SSRIs - UVB radiation

Answer 65

Incompetent one-way bicuspid valves in veins of legs prevent backflow--> chronic inflammation, fibrosis, etc. - With aging, valves compromised (incompetent)--> dilated veins Seen in 6-7% population > 50 years: - Superficial veins (most common): saphenous veins, tributaries - Deep veins: anterior and posterior tibial, peroneal, popliteal, deep femoral, superficial femoral, iliac veins - Perforating veins Causes: - Congenital, abnormal valves - Age-related - Trauma (injury, vein stripping) - Venous thrombosis Symptoms: - Throbbing, burning, aching, heaviness, restless legs, leg fatigue Clinical manifestations: - Spider veins - Varicose veins - Stasis dermatitis (often confused with bilateral cellulitis) - Acroangiodermatitis of Mali (tumor-like veins) - Stasis ulcers - Lipodermatosclerosis (loss of hair, shiny scar-like skin) Treatment: - Compression - Sclerotherapy - Vein stripping with ligation - Valvuloplasty

Answer 66

TEN= acute widespread blistering eruption caused by drugs Culprits: - Antibiotics (bactrum, sulfonamides, ampicillin) - Anticonvulsants (phenytoin) - Allopurionol - NSAIDs Clinical: - Eruption preceded by morbilliform, generalized erythema - Nikolsky sign (blister expands with physical manipulation) - Mucositis - Diff diagnosis: staph scalded skin Treatment: - Supportive care (similar to burn pt: fever, hyperthermia, hypotension due to volume losses, tachycardia, pneumonia, sepsis) - Corticosteroids (only if early) - High dose IVIG Mortality= 25-50%

Answer 67

Blistering disease of skin due to immune-complex deposition in papillary dermis - More common in N. Europeans - Age of onset= 2nd-3rd decade - 20% of patients have celiac disease, over 90% have GI findings on endoscopy Clinical findings: - Herpetiform (grouped) vesicles symmetrically distributed on knees, elbows, forearms, buttock, scalp (rare) - May have crusted lesions with non-specific pruritis (like atopic dermatitis, scabies, drug eruption) Pathogenesis: 1. Circulating antibodies including IgA to: - Gliadin (antigen= wheat protein) - Reticulum - Smooth muscle endomysium - Tissue transglutaminase 2. Deposition of immune-complexes in mucosa of: - Small intestine (malabsorption symptoms) - Papillary dermis of skin (blistering) Diagnosis: 1. Antigens in skin: - epidermal transglutaminase - antigen-antibody complex deposits in skin - NON-circulating 2. Skin biopsy - lesional skin forhisto: subepidermal bulla with neutrophils - Peri-lesional skin for immunofluorescence study (granular IgA deposition) 3. Tissue transglutaminase Ab in serum 4. DQ2/DQ8 HLA testing - high prevalence in normal population (low positive predictive value) - High negative predictive value Treatment: - Gluten-free diet (slow improvement) - Dapsone (anti-neutrophilic for skin symptoms) - Monitor for enteropathy-associated T-cell lymphoma (due to constant antigenic stimulation); DQ2 homozygotes at high risk

Answer 68

PCT= most common cause of all porphyria 2 forms: 1. Sporadic (80-90%): - Decreased activity of uroporphyrinogen decarboxylase in liver secondary to underlying liver disease 2. Familial (10-20%): - Decreased activity of uroporphyrinogen decarboxylase in all tissues, including erythrocytes * * Most gene carriers asymptomatic until sufficient liver damage occurs Preciptiators: - Male, over 40, alcohol - EtOH, estrogen, hexachlorobenzene, iron overload - Iron overload (in PCT)-_> decreased uroporphyrinogen decarboxylase activity Pathogenesis: - Uroporphyrinogen decarboxylase deficiency--> accumulation of photoactive molecules that absorb visible violet light - Photo-excited porphyrins in skin--> oxidative damage--> photosensitivity reactions Clinical manifestations: - Photosensitivity - Blisters and skin fragility on sun-exposed sites, dorsa of hands and feet, face - Shiny skin - Hypertrichosis (Hairy face) Associated disease: - Hep B, C - HIV - Beta-thal - Hemachromatosis - Hepatocellular carinoma - EtOH Diagnosis: - Skin biopsy (pauci-inflammatory subepidermal bulla= epidermal lifting with no inflammation, necrosis) - Fresh urine (pink to orange-red with Wood's lamp) - Porphyrin analysis (24 hr urine, plasma, stool) Treatment: - Phlebotomy (decrease Fe overload) - Hydroxycholorquine (anti-malarial) - Block sun exposure

Answer 69

Occurs at any age, generally young, healthy adult women - Tender erythematous nodular eruption (pre-tibial) - May be accompanied by arthralgia (ankles, knees, wrists) - Septal panniculitis Hypersensitivity reaction associated with other diseases, but 1/2 idiopathic - Infections (strep, viral, fungal) - IBD - Drugs (OCP, sulfonamides, penicillin) Usually self-limited Treatment: - Oral corticosteroids, SSKI (super-saturated potassium iodide)

Answer 70

Unknown pathogenesis Morphology: - Plaques with violaceous brown border, atrophic center - Histiocytes, lymphocytes, plasma cell around blood vessels Epidemiology: - 0.3% diabetic patients have NLD - 22-65% with NLD have diabetes - Avg age= 30 years No effective treatment: - intralesional steroids - doxycycline (anti-granulomatous)

Answer 71

ex: Henoch-Scholein Purpura, Behcet's disease Immune-complex deposition mediated destruction of post-capillary venules Skin= most common involved organ - Purpuric papules (palpable purpura) - Urticarial papules, purpuric macules, vesicles/bullae, pustules, ulcers - Distal areas, classically bilateral legs Skin lesions my have systemic signs/symptoms: - Fever, joint pain, abdominal pain, bloody urine - may indicate internal organ involvement Manifestation of diseases/meds: - Connective tissue diseases - Viral, bacterial infections - Malignancies, lymphomas/leukemias 50% cases are idiopathic Workup: CBC, BUN, Cr, LFT, ESR, RF, ANA, Antistreptolysin, cryocrit, serum protein electrophoresis, hepatitis serologies Histo: - Neutrophils - Extravasated RBCs - Fibrin deposition around vessels ``` Treatment: NSAIDs, antihistamines Colchicine Antibiotics (Dapsone) Corticosteroids ```

Answer 72

Form of LCV: - viral or bacteria-induced - frequently involves joint, GI tract, kidneys - Children 3-10 years of age - Immune complex consists of IgA (vs IgG) High risk of ESRD: - Serial urinalysis to detect kidney involvement (hematuria)

Answer 73

Necrotizing neutrophilic vasculitis of small to med-sized vessels with predilection for GI tract, liver, kidneys, skin Symptoms: - Fevers, arthralgia, myalgia, abdominal pain, mononeuritis multiplex, renovascular HTN, microaneurysms, NO pulmonary involvment - Skin involved in 50% cases, livedo reticularis, plaques, nodules, ulcerations - 10% skin-limited Associated with: - Hep B (7%) - Hep C, HIV, strep, CMV, parvo B19 - Most cases idiopathic Presentation: - ulceration - dispigmentation: retiform purpura on legs- worrisome vasculitis/vasculopathy (non-blanching) - nodules

Answer 74

APL syndrome seen in women (80%) ~42 years old 1-5% young, health people have Antiphospholipid antibodies without consequences - Occur with other conditions; IgM are present at low levels (infections, cancer, drugs, hemodialysis) Group of heterogeneous antibodies: 1. Lupus anticoagulant - dilute Russell's viper venom time (PTT prolongation) - Corrects with addition of excess phospholipid/platelets 2. Anticardiolipin - ELISA on cardiolipin-coated platelet in presence of bovine serum 3. anti-beta2 glycoprotein antibodies (phospholipid binding proteins) - ELISA for coated platelets Diagnostic criteria: - Anticardiolipin or lupus anticoagulant antibodies 2+ times, 6 weeks apart - Clinical: vascular thrombosis +/- pregnancy complications Clinical classfication: - Primary: no associated disease (90%) - Secondary: SLE, RA Presentation: 1. Thrombosis of large and small vessels of both arteries and veins of any organs - deep venous thrombosis= most common - arterial thrombosis= strokes and TIA 2. Complications - pulmonary emboli - emboli of vegetation of cardiac valves - gynecologic: miscarriages usually during fetal period, premature delivery 3. Acute involvement of small vessels= similar to HUS, TTP - results in loss of organ function - Catastrophic antiphospholipid syndrome Cutaneous manifestations: - superficial thrombophlebitis - ulcers - livedo reticularis - acrocyanosis Treatment: - High intensity warfarin therapy (INR: 3.1-4.0) - Plasmapheresis and/or IVIG for refractory and “catastrophic” cases

Answer 75

``` infections malignant neoplasms vasculitis vascular insufficiency diabetic ulcers pyoderma gangrenosum calciphylaxis factitial ulcers ``` Work-up: Skin biopsy, swab culture, tissue culture

Answer 76

Diagnosis of exclusion Chronic suppurative inflammation resulting in tissue destruction Size and depth of the ulcers vary Associated diseases: - inflammatory bowel disease, especially ulcerative colitis - IgA monoclonal gammopathy - leukemia

Answer 77

Almost exclusively seen in patients with end-stage renal disease - Progressive calcification of small arteries result in ischemic necrosis of the skin - No effective treatment - High mortality rate-60 to 80% due to sepsis Histo: fractured blood vessel lining= calcification Treatment: difficult (already sick with dialysis

Answer 78

Underlying causes: Multiple myeloma and Waldenstrom’s macroglobulinemia - Amyloid consists of immunoglobulin light chains - Deposition may occur in any organs Skin and mucosal lesions - deposits in vessels result in purpuras (leaky vessels) - pink translucent papules and nodules macroglossia Classic sign: carpal tunnel syndrome and purpura (e.g. raccoon eye sign, pinch purpura) Diagnosis: 1. Skin biopsy - amorphous pink homogeneous material - stains with Congo red, which turns bright green fluorescence upon polarization 2. Rectal mucosal biopsy 3. Abdominal subcutaneous fat aspirate

Answer 79

Usually widespread eruption of yellowish papules - High triglcyeride level due to lipoprotein lipase deficiency, less often seen in patients with diabetes and patients on retinoids (accutaine, isotretinoins) - Skin biopsy shows lipid laden foamy histiocytes and interstitial deposition - Complications include pancreatitis and atherosclerosis

Answer 80

The most common form of T-cell lymphoma of the skin (NOT fungus!) Clinical stages include patch, plaque, nodular, tumor, and erythrodermic stages - Unlike psoriasis, which has flares and remission, this is continuous Most patients have patches for a lifetime and a few will have progressive disease resulting in death Pathogenesis: - Neoplastic proliferation of T-helper cells (CD4+) - Sézary cells (CD4+ cells with convoluted nuclei) are found in the blood in the erythrodermic stages Treatment will depend on the clinical manifestation, but the disease is incurable

Answer 81

Virally induced (HHV8) vascular hyperplasia rather than a neoplasia - Metastatic vs multifocal - AIDS defining disease (HHV8 infection accompanied by immune suppression to manifest) In immunocompromised individuals, it can be found in the skin and in any visceral organs, especially: - GI bleeding, pulmonary hemorrhage, lymph nodes 4 types: 1. Epidemic AIDS related - Usually related to low CD 4 count 2. Classic/ sporadic - Elderly Mediterranean/ Eastern European men - Seen in lower extremity - NOT immune compromised 3. Immunocompromised - Transplantation - Sirolimus (mTOR inhibitor- can cause OR treat in transplant patients), anti-VEGF, antiangiogenesis 4. Endemic/ African - Indolent or aggressive - Lymphadenopathic form in children Manifestations: Patch, plaque, nodular, exophytic, infiltrative, lymphadenopathic Histology - Spindle cell proliferation of endothelial cells with slit-like vascular spaces, extravasated RBCs - May be more lymphatic in origin than vascular by staining patterns

Answer 82

Paraneoplastic syndrome (remote effect from primary neoplasm- paraneoplastic pemphigus, necrolytic migratory erythema, erythema gyratum repens) - Acute febrile neutrophilic dermatosis Characterized by: - tender edematous erythematous papules and plaques - accompanied by fever and peripheral neutrophilia Skin biopsy: - band like infiltrate of neutrophils in the dermis with edema of the papillary dermis - no leukocytoclasis of neutrophils (no dust) Associated with: - acute myeloid leukemia - drugs-GMCSF (neutrophil maturation) - pregnancy - inflammatory bowel diseases - upper respiratory infections Treatment: - Corticosteroids (treat infection/ malignancy first)

Answer 83

Velvety hyperpigmentation usually on the neck - Most frequent association is obesity - Rarely, association with adenocarcinoma of the GI tract has been described

Answer 84

Recurrent hyperpigmented patches with repeated use of drug - Can also see central necrosis in patch--> bullae Tx: stop offending agent Common offenders: barbiturates, tetracyclines, pseudoephedrine, nsaids/naproxen, erythromycin, sulfonamides, phenolpthalein

Answer 85

Generalized rash maculopapular with no malaise - Reaction to drugs started in past 2-3 weeks (unless previously exposed, could be 24-48 hours) Tx: identify and stop offending agent, topical steroids, +/- prednisone taper Workup: Monitor for evolution, mucosal involvement, onset ( avg =3 weeks) * CAN treat patient with same drug again (non life-threatening reaction); sensitize patient to drug in ICU

Answer 86

Drug eruption + Eosinophilia and Systemic symptoms - Severe drug rash Clinical: - widespread morbilliform eruption - oral mucosa (usually one site only) - avg time to onset 21 days Eosinophilia- ~50% cases Systemic Involvement- - elevated LFTS, - renal insufficiency, - hematologic abnormalities, - fever - LAD HHV6 association Common offenders: allopurinol, dapsone, phenytoin

Answer 87

Rash: erythematous macules coalescing into vesicles denuding Pathogenesis: extensive keratinocyte death via apoptosis Mucosal Involvement: 3 sites Body surface area Involvement % SJS: < 10% ; 10% < SJS/TEN 30% involvement Treatment: Derm Consult. - Identify and stop offending agent. - 95% cases due to drug (NSAIDS, Antibiotics (sulfas), Anticonvulsants) - No good treatment; - IVIg vs IV corticosteroids early Supportive care. Transfer to burn unit. Opthalmology consult. Prognosis: high mortality rate: 5% (SJS), 35% (TEN) Risk? - AIDS- 1000x fold increase - Slow acetylators - Immunocompromised - Brain tumor patients on anti-epileptics with radiation therapy * *screen for HLA-B*1502 in Asian patients to be prescribed carbamazepine

Answer 88

Even stupid drugs like hormones induce acne vulgaris: - EGFR inhibitors, - steroids, - dilantin, - lithium, - halogens, - INH, - ACTH, - Vitamins

Answer 89

- Epidermis forms as single layer in first weeks of embryogenesis - Neural crest elements (melanocytes) migrate to the epidermis (8th week) - Adnexa (hair, eccrine glands) form around 12th week as invaginations from the epidermis into the dermis - Sebaceous and apocrine glands sprout from follicular epithelium

Answer 90

Epidermis: stratum basale, spinosum, granulosum, lucidum, corneum - keratinocytes (adnexae) - melanocytes - Langerhans cells - Merkel cells (sensory) Dermis: - fibroblasts (elastic fibers) - Bone- marrow derived cells: Mast cells, B/T lymphocytes - Vessels (endo and pericytes) - Neural tissue Subcutaneous fat

Answer 91

APC (antigen-presenting cells) seen in epidermis) | - Characteristic Birbeck granules seen by electron microscopy

Answer 92

Angiocentric: around blood vessels - Urticaria, necrotizing vasculitis Spongiotic: increased fluid between keratinocytes (edema) - Allergic contact dermatitis Interface: Inflammation between epidermis and dermis - Lupus, contact dermatitis Vesiculo-bullous: blistering between epidermis and dermis (lifting) - Pemphigus Panniculitis: inflammation of subcutaneous fat - Erythema nodosum

Answer 93

Hyperplasia= Acanthosis - Psoriasis Dysplasia= atypical cell Neoplasia: benign and malignant 1. Benign Epidermal: - Seborrheic keratosis, Verruca 2. Pre-malignant Epidermal: Actinic keratosis 3. Malignant Epidermal: - Squamous cell carcinoma, Basal cell carcinoma 4. Benign Melanocytic: Melanocytic nevus 5. “Pre-malignant” Melanocytic: - Dysplastic/Atypical melanocytic nevus 6. Malignant Melanocytic: Melanoma

Answer 94

Solar elastosis: dense clumpy deposition of elastic fibers due to chronic sun damage Secondary amyloidosis: deposition of amyloid in skin Gout: deposition of birefringent uric acid crystals in dermis surrounded by granulomatous inflammation

Answer 95

Angiocentric skin changes Often young adults - Transient, lasts < 24 hrs - Non-purpuric wheals: Edematous, erythematous papule or plaque - Intensely pruritic (itchy) - Angioedema (deep) - Chronic forms: Urticarial vasculitis, Underlying disease Pressure - Dermatographism Type I hypersensitivity reaction - Immunologic (IgE-mediated): Mast cell degranulation, Release of histamine, prostaglandins - Non-immunologic: Mechanical (dermatographism), Temperature Pathophys: - Mast cells degranulate: release Histamine, prostaglandins, cytokines - -> Vascular dilation, Dermal edema, Endothelial activation - E-selectin/CLA binding - Angiocentric inflammation: Lymphocytes, neutrophils, eosinophils - No epidermal changes

Answer 96

Angiocentric skin changes Seen on Lower extremities - Palpable purpura= Red macules and papules with dot-like hemorrhage - Diascopy Limited to skin or systemic - Drugs, infection, connective tissue disease, underlying malignancy (Idiopathic in 50%) Henoch-Schönlein Purpura: seen after strep, viral illness (hematuria, abdominal pain, vasculitis mediated by IgA) Pathophys: - Infiltrate of neutrophils with fragmentation (nuclear “dust”) - Neutrophils chew thru fibrin in vessel walls and lumen (fibrinoid necrosis) - Hemorrhage due to vessel wall destruction (extravasation--> no blanching) - Positive DIF (direct immunofluorescence)

Answer 97

Form of Spongiotic dermatosis Pruritic, well-demarcated, erythematous, edematous papules and plaques with vesicle formation - Blisters in 1-2 days (Often linear lesions) - Epicutaneous contact (Poison ivy) - Scaly with chronicity Pathophys: - Type IV reaction= cell-mediated or delayed hypersensitivity - Antigen taken up by LC; LC to draining LN; present to T helper cells (MHC class II); becomes sensitized - Sensitization/challenge Histo: - Intercellular edema between keratinocytes: May form vesicles - Langerhans cells in vesicles - Perivascular infiltrate of lymphocytes, histiocytes, and eosinophils

Answer 98

Acanthotic process: hyperplasia of epidermis Erythematous plaques with silvery scale - Pustules (early) - Silvery scale (late) - Often symmetric, B/L: Elbow, knees, scalp, intertriginous, or generalized - Koebner phenomenon (damage--> lesion) - Auspitz sign (scale lifted--> bleeding) - HLA types : Cw6, B13, B17, Bw57 Psoriatic arthritis: seen in 5-8% of psoriasis patients - higher association with nail bed changes Histo: - “Regular” acanthosis - Parakeratosis - Neutrophils in scale - Loss of granular layer - Thin suprapapillary dermal plate - Prominent, dilated capillaries Pathogenesis: - Lymphocytes - Autoreactive T cell - Keratinocytes - Shortened epidermal cell cycle (28x nl production) - Vessel alterations: abnormal post-capillary vessels (dilated)

Answer 99

Interface changes in epidermis/dermis: Acute Form: - Facial erythema (malar rash) - Generalized erythema: Face, arms, neck, dorsal hands - Bullae Subacute Form - Psoriasiform - Annular vesicular Chronic Forms - Discoid lesions (5-10% have systemic disease) - Scaling, atrophic plaque - Hypo/hyperpigmentation - Follicular plugging - Alopecia - Panniculitis (50% have systemic disease) Discoid lupus (confined to skin, may develop systemic disease in 5-10%): - Localized to skin - Scaling atrophic plaques - Pigmentary changes - Telangiectasia - Alopecia - Follicular plugging Discoid lupus histology: - Hyperkeratosis (scale) - Epidermal atrophy= Lymphocytes attacking DEJ (interface) 1. Basal cell vacuolization due to interface 2. Necrotic keratinocytes (attacked by lymphocytes- pyknotic keratinocytes) 3. Pigment incontinence: dump melanin) - Superficial & deep perivascular & perifollicular: Follicular destruction - Dilated vessels - Positive ‘Lupus Band’= Granular IgG & C3 along thickened BM

Answer 100

Interface process Scaly, flat-topped, violaceous papules and plaques The 5 Ps: 1. Pink to purple 2. Pruritic 3. Polygonal 4. Papules 5. Plaques - Often wrists and ankles; Can occur anywhere including oral mucosa - Most cases idiopathic Histo: interface process= - Superficial band-like lymphocytic infiltrate (DEJ) - Necrotic keratinocytes - Hyperkeratosis - Hypergranulosis (wedge-shaped) - Irregular acanthosis - Saw-toothing of rete edges

Answer 101

Vesiculobullous process - Flaccid blisters; Easily ruptured - Erosions - Scalp, mucosa, intertriginous sites - Nikolsky sign: press lateral edge of blister, it will spread - Genetic predisposition - Commonly 40s-50s - Autoimmune disease Histo: - Blistering is Supra-basal (Tombstone pattern where basal keratinocytes still attached to dermis) - Acantholysis - Autoantibodies to Desmoglein 3--> destruction of Desmosomes * * vs Bullous pemphigous= entire epidermal layer lifts from dermis (no keratinocytes still resting on basement membrane= sub-basal) - Sub-epidermal blistering also seen in dermatitis herpetiformis

Answer 102

``` Panniculitis Tender erythematous nodules on extensor surfaces of lower legs - Dome-shaped, firm erythematous nodules - Self-limited, constitutional symptoms - Seen in young women ``` Immunologic response to foreign antigens - Can be due to distal infection (bacterial, viral, TB, fungi), drugs (sulfa, OCPs), sarcoid, IBD, tumors, etc. Histo: - Septal - Mostly chronic inflammation - Granulomas - Fibrosis - Mimicry: Infection, Factitia

Answer 103

Benign Epidermal neoplasia - Middle age and older - Oval or round “stuck on” papule or plaque - Flesh-colored to brown or black - Rough surface - Symmetrical, sharp margins - Sign of Leser-Trélat: multiple developed over short period of time--> underlying malignancy (release of growth factors) - Cause???- genetics, sun exposure, viral Histo: - Basaloid keratinocytes (follicular infundibulum): can grow up or invaginate - “Horn cysts”= invagination, gives rough appearance - No melanocytic derivation (keratinocytes!)

Answer 104

``` Benign epidermal neoplasia; Also, an infection (HPV)! - Seen at Sites of inoculation - Malignant transformation with immunosuppression - “Black dots” when peeled away Types: - Verruca vulgaris - Verruca palmaris/plantaris - Verruca plana - Condyloma acuminatum ``` HIsto: - Benign squamoid proliferation - Crown-like with vascularized spires; irritation--> bleeding--> black hemorrhagic spots - Koilocytosis: nucleus with glassy appearance (intranuclear virus)--> crinkled with halo - Keratohyaline granules within keratinocytes - HPV genome (typing): immunohistochem, FISH (high or low risk) - Cell-mediated immune response--> resolution (unless immune suppressed)

Answer 105

Pre-malignant epidermal changes: - Rough, scaling, reddish, ill-defined patch or plaque (can see cutaneous horn; may also be wart, carcinoma--> biopsy!) - Occurs on sun-exposed skin - UV-radiation induced keratinocyte DNA damage - Can spontaneously regress (up to 25%) - May progress to squamous cell carcinoma Histo: - Variable atypia; Less than full epidermal thickness - Hyperkeratosis - Parakeratosis - Solar elastosis - Chronic dermal inflammation

Answer 106

Malignant epidermal neoplasia - Scaling, erythematous to flesh-colored plaque or nodule - May be scaly, ulcerated or crusted - Occurs most often on sun-exposed skin (75% on head and neck) - Also on mucous membranes and sites of chronic injury (burn scars, irradiated sites, sinus tracts) 2nd most common skin cancer - UV radiation induced immunosuppression - Locally invasive Potential to metastasize - 2-5% of SCCs related to sun exposure - Up to 20% of SCCs arising in sites of chronic injury (draining sinuses, burn scars) and mucous membranes Histo: - Atypical keratinocytes - Growth into underlying dermis - Keratin pearls (altered, denser homogenous keratin formation) - Hyperkeratosis - Parakeratosis

Answer 107

Malignant epidermal neoplasia - Erythematous patch with central erosion/ulceration and rolled, pearly border OR a pearly papule or nodule - Telangiectasia - Seen on sun-exposed skin, most common on face - Most common skin cancer; most common human malignancy - Rarely metastasizes but may be locally aggressive Histo: - Enlarged basaloid keratinocytes (increased mitoses) - Individual cell necrosis= malignancy - Bud and extend into dermis - Peripheral palisading - Separation artifact on histologic preparation: due to mucinous stroma- washed away after processing

Answer 108

``` Benign melanocytic neoplasia - Most acquired after age 6 months and before age 35 Variable (but uniform) appearance: - Macular or papular - Brown to flesh-colored - Usually <5 mm in diameter ``` Junctional: present as nest in epidermal-dermal junction Compound: involves dermis and epidermis Dermal: neval cells only seen in dermis Histo: - Derived from neural crest origin - Maturation with descent into dermis

Answer 109

Melanocytic dysplasia - Controversial entitiy Abnormal: clinical appearance, architecture, cytology, stroma Isolated vs dysplastic nevi syndrome - Clinical phenotype - Personal and family history - 100% lifetime risk Premalignant potential, markers for increased melanoma risk Histo: - Irregular nests: bridging (nests coalesce), shoulder (epidermal component descends beyond dermis) - Cytologic atypia - Stromal fibrosis, lamellar fibroplasia (collagen, onion-skin layering around rete ridges)

Answer 110

TNM stage: 1. Measured Depth (mm)= thickness (#1 prognostic feature) 2. Ulceration (bad sign) 3. Mitotic rate (more mitoses, worse prognosis) - Clark level (obsolete finding- how deep) - TIL Response (tumor-infiltrating lymphocyte response= good prognosis) - Regression (bad prognosis: body eradicates part of melanoma) - Vascular Invasion (mets) - Microscopic Satellites (bad) - Gender (F fare better than M) - Site (extremities except hand and feet do better) - Stage (metastasized= prognostic indicator)