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Home , Dermatitis herpetiformis, Porphyria cutanea tarda

7/13/16

Conflict of Interest 2016 Internal Medicine Board Review I have no relevant conflicts to disclose.

Dermatology

Nina Botto/ Kanade Shinkai, MD PhD/ Lindy Fox, MD Assistant Professor of Department of Dermatology

University of California, San Francisco 1 2

• You also notice Case 1 – Erosions (fragility) • A 45 year old man – presents with painless – vesicles and bullae on – Milia his face and dorsal hands

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Case 1, Question 1 Case 1, Question 1 The most likely diagnosis is: The most likely diagnosis is: A. vulgaris A. B. Bullous B. Bullous impetigo C. Bullous C. D. cutanea tarda D. E. herpetiformis E.

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Case 1, Question 2 Case 1, Question 2 Porphyria cutanea tarda Porphyria cutanea tarda The underlying condition most likely to be The underlying condition most likely to be associated is: associated is: A. Hemochromatosis A. Hemochromatosis B. B. Hepatitis C C. Chronic renal insufficiency C. Chronic renal insufficiency D. Diabetes mellitus D. Diabetes mellitus E. NSAID use E. NSAID use

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Porphyria Cutanea Tarda (PCT) Porphyria Cutanea Tarda • Most common form of porphyria • Sun-exposed sites • 5th decade of life (dorsal hands, ears, • M (60%), F (40%) face) • Risk factors • Non-inflammatory bulla – HCV 85% • fragility – Hemochromatosis • Facial hypertrichosis – • Milia – Genetic predisposition • overload -> reduced • Hyperpigmentation

uroporphyrinogen decarboxylase activity 9 10

Porphyria Cutanea Tarda Case 2 Treatment • 43 yo Scandinavian male • Phlebotomy +/- erythropoetin • Pruritic and • Low dose vesicles on extensor surfaces and buttocks – 200 mg twice per week • No mucosal involvement • Sun avoidance/photoprotection • Weight loss, chronic , diarrhea

• Small bowel biopsy: shortening of intestinal villa 11 12

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Case 2, Question 1 The most likely diagnosis is: A. Pemphigus vulgaris B. Bullous impetigo C. Bullous pemphigoid D. Porphyria cutanea tarda E. Dermatitis herpetiformis

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Case 2, Question 1 Case 2, Question 2 The most likely diagnosis is: Dermatitis Herpetiformis A. Pemphigus vulgaris This condition is most closely associated with: B. Bullous impetigo A. Underlying lymphoma C. Bullous pemphigoid B. -sensitive enteropathy D. Porphyria cutanea tarda C. Autoimmune E. Dermatitis herpetiformis D. Diabetes mellitus E. No associated underlying condition

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Case 2, Question 2 Dermatitis Herpetiformis Dermatitis Herpetiformis • Symmetric, This condition is most closely associated with: erythematous vesicles A. Underlying lymphoma and papules in groups B. Gluten-sensitive enteropathy C. Autoimmune diseases • Intensely pruritic D. Diabetes mellitus • Distribution is a clue: E. No associated underlying condition – Elbows, knees, forearms, buttocks, scalp, neck

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Dermatitis Herpetiformis Dermatitis Herpetiformis Associated Diseases Diagnosis

• Associated with gluten-sensitive enteropathy Test Mode Result in DH Notes H&E Collections of DIF(+ ) • Increased risk of GI lymphoma DIF at granular IgA upper dermal-epidermal • Thyroid diseases in 20% junction – hypothyroidism #1 IgA tissue ELISA, blood Sensitivity 90% Higher false (+), – acute autoimmune thyroiditis Specificity 95% confirm with anti- – hyperthyroidism endomysial Ab

• Other: pernicious anemia, Addison’s IgA anti- IF, blood (+) 70-90% Antigen is tissue endomysial Ab transglutaminase

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Dermatitis Herpetiformis Case 3 • Healthy 20 yo college student Treatment • Pruritic eruption x 10 days • Gluten free diet • (50-300 mg daily) – rapid response • Does not respond to topical or systemic steroids

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Case 3, Question 1 Case 3, Question 1 The most likely diagnosis is: The most likely diagnosis is: A. A. Psoriasis B. B. Pityriasis rosea C. Secondary C. Secondary syphilis D. Subacute cutaneous D. Subacute cutaneous lupus E. E. Tinea versicolor

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Pityriasis Rosea Case 4 • Common • 48 yr old man • Herald patch: 1 week • Facial x 3 months earlier, larger plaque • Increasing • Annular scaly plaques • Difficulty stocking • Central trunk and back overhead shelves (Christmas tree pattern)

• Mimics the rash of secondary syphilis – CHECK RPR

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Case 4, Question 1 The lab test most likely to be abnormal is: A. ESR B. Anti-smith C. Rheumatoid factor D. Serum creatine kinase E. Anti-dsDNA

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Case 4, Question 1 Dermatomyositis The lab test most likely to be abnormal is: • Proximal muscle weakness

A. ESR • Characteristic skin findings B. Anti-smith antibody – Heliotrope: peri-orbital , violaceous rash @ eyelids – Gottron’s papules: flat, violaceous @ MCP, PIP, DIP joints C. Rheumatoid factor – Photosensitive rash, shawl sign D. Serum creatine kinase – Skin biopsy: similar to lupus (vacuolar interface + mucin) E. Anti-dsDNA • Lab tests: – Elevated CK or aldolase – Muscle biopsy, electromyography, MRI – ANA positive in 60-80% – Anti-Jo antibody associated with interstitial lung disease 31 32

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Case 4, Question 2 Case 4, Question 2 Dermatomyositis Dermatomyositis • In an adult female patient with • In an adult female patient with dermatomyositis, which is the most dermatomyositis, which is the most important test to evaluate for an important test to evaluate for an associated malignancy? associated malignancy? A. Thyroid scan A. Thyroid scan B. Mammogram B. Mammogram C. Colonoscopy C. Colonoscopy D. Upper endoscopy D. Upper endoscopy E. Pelvic ultrasound E. Pelvic ultrasound 33 34

Dermatomyositis Next case: Case 5 Paraneoplastic Associations • Dermatomyositis is associated with underlying malignancy in 32% of adult patients – Risk highest > age 45, especially men • Women: ovarian cancer • Men: lung cancer • Asians: hepatomas, esophageal adenoCA

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Subacute Cutaneous LE Case 5 (SCLE) • 24 YO M with a sudden onset rash that • Women aged 15-40 began on a beach vacation. Which is most • 50% meet ARA criteria for SLE, only 10% likely diagnosis? severe – A) • Renal or CNS disease rare = good prognosis – B) secondary syphylis • Consider drug-induced form – C) subacute cutaneous • 80% ANA positive – D) • Positive Ro/SSA – Neonatal heart block is risk • Photosensitive

37 – Ro correlates with photosensitivity 38

Subacute Cutaneous LE Case 6 Skin Lesions • Papulosquamous: • 55 yr old male Resembles psoriasis • COPD, HTN, h/o psoriasis • Annular • Fever, shaking chills, and • Sun-exposed areas diffuse (erythroderma) • Face, V-neck chest, and back • Heals without scarring • Meds: • (unlike discoid LE) – ACE inhibitor x 3 months – 1 week of pulsed prednisone with rapid 39 40 taper for COPD flare

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Case 6, Question 1 The most likely diagnosis is: A. due to ACE inhibitor B. due to non-small cell lung cancer C. Sézary syndrome (cutaneous T-cell lymphoma) D. Flare of psoriasis due to prednisone taper E. Staphylococcal Scalded Skin Syndrome

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Case 6, Question 1 Pustular Psoriasis The most likely diagnosis is: • Commonly drug-induced A. Drug eruption due to ACE inhibitor • Corticosteroid taper B. Paraneoplastic syndrome due to non-small cell lung cancer • Psoriasis flare + pustules C. Sézary syndrome (cutaneous T-cell lymphoma) • Can be life threatening D. Flare of psoriasis due to prednisone taper – High cardiac output state E. Staphylococcal Scalded Skin Syndrome – Electrolyte imbalance – Respiratory distress – Temperature dysregulation

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Psoriasis Comorbidities Psoriasis • Recent evidence links severe psoriasis with – Arthritis – Cardiovascular disease (including MI) – Hypertension – Obesity – Diabetes – Metabolic syndrome – Malignancies • Lymphomas, SCCs, ? Solid organ malignancies – Higher mortality – Poor quality of life 45 46

Psoriasis Aggravators

• – Systemic steroids – Beta blockers – Lithium – Hydroxychloroquine • Strep infections (children, guttate psoriasis) • Trauma (friction, sunburn) • HIV 47 48

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Treatment for Psoriasis Case 7 • Topical therapy • 42 yo HIV+ male admitted to ICU – Steroid ointment (start mid-potency) • Severely hypotensive à IV fluids, norepinephrine – Calcipotriene ointment • ?Sepsis à are started • Phototherapy • History of taking TMP/SMX for UTI – PUVA: psoralens + UVA • Systemic therapy • 24 hrs after admission: – Acitretin (oral retinoid) • febrile – Methotrexate, cyclosporine • rash, rapidly progressive – Biologics • skin is painful • etanercept, infliximab, adalimumab (TNF alpha inhibitor) • gritty sensation in eyes • ustekinumab (IL-12, IL-23 blockade) • oral pain, difficulty swallowing 49 50 **Systemic steroids are NOT on this list!**

Case 7, Question 1 The most likely diagnosis is: A. Drug Eruption B. Staphylococcal Scalded Skin Syndrome C. Autoimmune Blistering Disorder D. Toxic Shock Syndrome E. Severe viral

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Case 7, Question 1 Case 7, Question 2 The most likely diagnosis is: A. Drug Eruption All of the following are red flags of a serious B. Staphylococcal Scalded Skin Syndrome drug eruption except: C. Autoimmune Blistering Disorder A. Oral (mucocutaneous) involvement B. Morbilliform eruption D. Toxic Shock Syndrome C. Vesicle/ Bullae E. Severe viral exanthem D. Target lesions E. Facial edema Toxic epidermal necrolysis (TEN)

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Case 7, Question 2 Case 7, Question 3

All of the following are red flags of a serious All of the following are signs/symptoms of a drug eruption except: drug hypersensitivity reaction except: A. Oral (mucocutaneous) involvement A. B. Morbilliform eruption B. Lymphadenopathy C. Vesicle/ Bullae C. Elevated function tests D. Target lesions D. Hypertension E. Facial edema E. Facial edema

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Drug eruptions: Case 7, Question 3 Simple or Complex All of the following are signs/symptoms of a drug hypersensitivity reaction except: Simple Complex A. Eosinophilia Morbilliform drug eruption Drug hypersensitivity reaction B. Lymphadenopathy Stevens-Johnson (SJS) C. Elevated liver function tests Toxic epidermal necrolysis D. Hypertension (TEN) E. Facial edema

Minimal systemic symptoms Systemic involvement

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Morbilliform (Simple) Drug Anticonvulsant Hypersensitivity Eruption Reaction • common • erythematous macules, papules • pruritus • no systemic symptoms • begins in 2nd week

• treatment: -D/C med if severe -symptomatic treatment: diphenhydramine, topical steroids 59 Facial edema is key to the diagnosis of drug hypersensitivity reactions60

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Hypersensitivity Reactions Hypersensitivity Reactions Clinical features (General) • Morbilliform rash + visceral involvement • Rash • Late onset: 2- 6 weeks after started • Fever • Classic associations • Pharyngitis – Aromatic anticonvulsants THESE CROSS-REACT • Hepatitis • phenobarbital, carbamazepine, phenytoin, lamotrigine – Allopurinol • Myocarditis – Dapsone • Hematologic abnormalities – NSAIDs – eosinophilia – Sulfonamides – atypical lymphocytosis, lymphadenopathy – Anti-retrovirals • Facial edema • Treatment: 61 – Must stop medication (cannot treat through) 62 – Prednisone (1-2mg/kg/day)

Stevens-Johnson Syndrome (SJS) Toxic Epidermal Necrolysis (TEN)

• Widespread bullae • Skin pain > pruritus • Erythema -> bullae -> denuded dermis

• Medical emergency: call dermatology immediately

>2 mucosal sites Target lesions 63 64

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Toxic Epidermal Necrolysis (TEN) SJS/TEN: Emergency Management • Stop all unnecessary medications – The major predictor of survival and severity of disease • Treatment – Systemic • Check for Mycoplasma- 25% of SJS in pediatric patients • Controversial –SJS: high dose corticosteroids –TEN: IVIG 0.5-1g/kg/d x 4 days – Refer to burn unit or ICU early • Reduces risk of infection and reduces mortality to 5% • Call Ophthalmology 65 66

Case 8 Case 8, Question 1 • 37 yo woman with inflammatory bowel disease • Rapidly progressive, painful ulceration • The most appropriate treatment for this • 3 days after bumping her leg on a chair disorder is A. Systemic steroids B. Intravenous antibiotics C. Surgical debridement D. Compression dressing E. Wet to dry dressings

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Case 8, Question 1 Pyoderma Gangrenosum

• The most appropriate treatment for this • Non-infectious, inflammatory disorder is • Diagnosis of exclusion (must r/o infection) A. Systemic steroids • Rapidly progressive (days) ulcerative process B. Intravenous antibiotics • Begins as a small pustule C. Surgical debridement • Undermined violaceous border D. Compression dressing • Triggered by trauma (pathergy) (surgical E. Wet to dry dressings debridement, attempts to graft)

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Pyoderma Gangrenosum Pyoderma Gangrenosum Treatment • 50% idiopathic • Treatment of underlying disease may not help PG

• Associations: • Topical therapy: – Superpotent topical steroids or tacrolimus – IBD(1.5%-5% of IBD patients get PG) • Systemic therapy: – Rheumatoid arthritis – Systemic steroids – Seronegative arthritis – Cyclosporine, tacrolimus – Hematologic – Mycophenolate mofetil abnormalities – Thalidomide

71 – TNF-inhibitors (infliximab) 72

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Case 9 Case 9,Question 1

• 52 yr old Caucasian The most significant prognostic indicator for male is: • Enlarging mole on his back A. Anatomic location of melanoma • Family history – B. Diameter of melanoma brother recently C. Positive family history in a first degree diagnosed with relative melanoma, mother died of metastatic D. Thickness of melanoma melanoma E. History numerous childhood blistering sunburns

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Case 9,Question 1 Diagnosis of Melanoma

The most significant prognostic indicator for • Prognosis is DEPENDENT on the depth of melanoma is: lesion (Breslow’s depth) A. Anatomic location of melanoma – < 1mm thickness is low risk B. Diameter of melanoma – > 1mm Consider sentinel biopsy C. Positive family history in a first degree relative • If melanoma is on the differential, complete D. Thickness of melanoma excision or full thickness incisional biopsy is indicated E. History numerous childhood blistering sunburns

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Malignant Melanoma Malignant Melanoma

• Asymmetry • Treatment: many new molecular targets • Border • Confer survival • Color • B-Raf inhibitors • Diameter (>6mm) • PD-1 inhibitors • Evolution

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Skin Cancer: Case 10 Case 10

• 65 year old man s/p • Which is the most renal transplant likely diagnosis? • Rapid growth of nodule • A) SCC on leg • B) BCC • C) Melanoma in situ • D) Pyoderma gangranosum

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Skin Cancer in Organ Transplant Skin Cancer in Organ Transplant Recipients Recipients • Skin cancer: #1 common malignancy • Risk Factors • Incidence increases with survival time post transplant – Increased age at transplant (BCC) • Important cause of morbidity, mortality – Exposure to UV radiation • 90% are nonmelanoma skin cancer – Amount of immunosuppression (SCC) – Squamous cell carcinoma (SCC) – Fair skin (BCC) • 65x increased risk – Personal history of AK, NMSC, melanoma – Basal cell carcinoma (BCC) – Heart > kidney > liver transplants • 10x increased risk – HPV infection – Melanoma Arch Dermatol 2004, 140:1079. • 2x increased risk Derm Therapy. 2005; 18: 12-18 Traywick and O’Rielly. Derm Therapy. 2005; 18: 12-18

Skin Cancer in Organ Transplant Case 11 Recipients • To reduce skin cancer risk: • 30 yr old Asian female – Reduce immunosuppression to minimum • Fevers, malaise, arthralgia required • Prior history of lower extremity with recent 10 day – Photoprotection course of cephalexin – Acitretin (25 mg daily) may reduce rate of • Meds: OCPs SCC development • ROS: intermittent abdominal pain and loose stool • Refer organ transplant patients to a • Family history: inflammatory bowel disease dermatologist for regular skin checks

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Case 11, Question 1 Case 11, Question 1

The most likely diagnosis is: The most likely diagnosis is: A. A. Erythema multiforme B. Cellulitis resistant to cephalosporins B. Cellulitis resistant to cephalosporins C. Pretibial myxedema C. Pretibial myxedema D. D. Erythema nodosum E. Sweet’s syndrome E. Sweet’s syndrome

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What about if lesions are more Erythema Nodosum: Associations purpuric, raised? • Common • Uncommon – Idiopathic (35-55%) – Yersinia • 37 yo man with HCV – Behçet's – Infection: STREP – Sweet’s syndrome – fever, joint pain, and rash • URI, Mycoplasma, TB – Pregnancy • Cocci (+ prognosis) – Malignancy • Rare – Brucellosis – Drugs – Meninigococcus/gonococcus • OCPs, sulfonamides, – E. coli PCN, halides – Pertussis – Syphilis – – – Cat Scratch – Chlamydia – Blastomycosis – IBD (Crohns > UC) – – HIV 87 88

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Case 11

• A) leukocytoclastic • B) meningococcemia • C) erythema multiforme • D) angiosarcoma

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Leukocytoclastic Vasculitis Differential Diagnosis • Infection • Mixed cyroglobulinemia – Post strep GN (HCV) – Hepatitis B • Malignancy associated – SBE – CLL • Hypersensitivity – Multiple myeloma – Henoch-Schönlein purpura – Lymphoma – Serum sickness – Hodgkin’s disease – Medication • ANCA associated • Rheumatic disease vasculitis – SLE – Wegeners granulomatosis – RA – Microscopic polyangiitis – Sjögren’s syndrome Good Luck! 91

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