Conflict of Interest 2016 I have no relevant conflicts to disclose. - PDF document

7/13/16 Conflict of Interest 2016 I have no relevant conflicts to disclose. Internal Medicine Board Review Dermatology Nina Botto/ Kanade Shinkai, MD PhD/ Lindy Fox, MD Assistant Professor of Dermatology Department of Dermatology University of California, San Francisco 1 2 • You also notice Case 1 –Erosions (fragility) –Hypertrichosis • A 45 year old man presents with painless –Hyperpigmentation vesicles and bullae on –Milia his face and dorsal hands 3 4 1

7/13/16 Case 1, Question 1 Case 1, Question 1 The most likely diagnosis is: The most likely diagnosis is: A. Pemphigus vulgaris A. Pemphigus vulgaris B. Bullous impetigo B. Bullous impetigo C. Bullous pemphigoid C. Bullous pemphigoid D. Porphyria cutanea tarda D. Porphyria cutanea tarda E. Dermatitis herpetiformis E. Dermatitis herpetiformis 5 6 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. Hepatitis C B. Hepatitis C C. Chronic renal insufficiency C. Chronic renal insufficiency D. Diabetes mellitus D. Diabetes mellitus E. NSAID use E. NSAID use 7 8 2

7/13/16 Porphyria Cutanea Tarda (PCT) Porphyria Cutanea Tarda • Sun-exposed sites • Most common form of porphyria • 5 th decade of life (dorsal hands, ears, face) • M (60%), F (40%) • Non-inflammatory bulla • Risk factors – HCV 85% • Skin fragility – Hemochromatosis • Facial hypertrichosis – Alcoholism • Milia – Genetic predisposition • Hyperpigmentation • Iron overload -> reduced uroporphyrinogen decarboxylase activity 9 10 Porphyria Cutanea Tarda Case 2 Treatment • 43 yo Scandinavian male • Phlebotomy +/- erythropoetin • Pruritic papules and vesicles on extensor • Low dose hydroxychloroquine surfaces and buttocks – 200 mg twice per week • No mucosal involvement • Sun avoidance/photoprotection • Weight loss, chronic abdominal pain, diarrhea • Small bowel biopsy: shortening of intestinal villa 11 12 3

7/13/16 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 13 14 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. Gluten-sensitive enteropathy D. Porphyria cutanea tarda C. Autoimmune diseases E. Dermatitis herpetiformis D. Diabetes mellitus E. No associated underlying condition 15 16 4

7/13/16 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 • Intensely pruritic C. Autoimmune diseases D. Diabetes mellitus • Distribution is a clue: E. No associated underlying condition – Elbows, knees, forearms, buttocks, scalp, neck 17 18 Dermatitis Herpetiformis Dermatitis Herpetiformis Associated Diseases Diagnosis • Associated with gluten-sensitive enteropathy Test Mode Result in DH Notes Skin biopsy H&E Collections of DIF(+) • Increased risk of GI lymphoma DIF neutrophils at granular IgA upper dermal-epidermal dermis • Thyroid diseases in 20% junction – hypothyroidism #1 IgA tissue ELISA, blood Sensitivity 90% Higher false (+), – acute autoimmune thyroiditis transglutaminase Specificity 95% confirm with anti- endomysial Ab – hyperthyroidism • Other: pernicious anemia, Addison’s disease IgA anti- IF, blood (+) 70-90% Antigen is tissue endomysial Ab transglutaminase 19 20 5

7/13/16 Dermatitis Herpetiformis Case 3 • Healthy 20 yo college student Treatment • Pruritic eruption x 10 days • Gluten free diet • Dapsone (50-300 mg daily) – rapid response • Does not respond to topical or systemic steroids 21 22 Case 3, Question 1 Case 3, Question 1 The most likely diagnosis is: The most likely diagnosis is: A. Psoriasis A. Psoriasis B. Pityriasis rosea B. Pityriasis rosea C. Secondary syphilis C. Secondary syphilis D. Subacute cutaneous lupus D. Subacute cutaneous lupus E. Tinea versicolor E. Tinea versicolor 23 24 6

7/13/16 Pityriasis Rosea Case 4 • 48 yr old man • Common • Facial rash x 3 months • Herald patch: 1 week • Increasing fatigue earlier, larger plaque • Difficulty stocking • Annular scaly plaques overhead shelves • Central trunk and back (Christmas tree pattern) • Mimics the rash of secondary syphilis – CHECK RPR 25 26 27 28 7

7/13/16 Case 4, Question 1 The lab test most likely to be abnormal is: A. ESR B. Anti-smith antibody C. Rheumatoid factor D. Serum creatine kinase E. Anti-dsDNA 29 30 Dermatomyositis Case 4, Question 1 • Proximal muscle weakness The lab test most likely to be abnormal is: A. ESR • Characteristic skin findings – Heliotrope: peri-orbital edema, violaceous rash @ eyelids B. Anti-smith antibody – Gottron ’ s papules: flat, violaceous @ MCP, PIP, DIP joints C. Rheumatoid factor – Photosensitive rash, shawl sign – Skin biopsy: similar to lupus (vacuolar interface + mucin) D. Serum creatine kinase 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 8

7/13/16 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 35 36 9

7/13/16 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% severe likely diagnosis? • Renal or CNS disease rare = good prognosis – A) mycosis fungoides • Consider drug-induced form – B) secondary syphylis • 80% ANA positive – C) subacute cutaneous lupus erythematosus • Positive Ro/SSA – D) tinea corporis – Neonatal heart block is risk • Photosensitive – Ro correlates with photosensitivity 37 38 Subacute Cutaneous LE Case 6 Skin Lesions • 55 yr old male • Papulosquamous: • COPD, HTN, h/o psoriasis Resembles psoriasis • Fever, shaking chills, and • Annular diffuse erythema • Sun-exposed areas (erythroderma) • Face, V-neck chest, and back • Meds: • Heals without scarring – ACE inhibitor x 3 months • (unlike discoid LE) – 1 week of pulsed prednisone with rapid 39 40 taper for COPD flare 10

7/13/16 Case 6, Question 1 The most likely diagnosis is: A. Drug eruption due to ACE inhibitor B. Paraneoplastic syndrome 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 41 42 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 • Psoriasis flare + pustules lung cancer C. Sézary syndrome (cutaneous T-cell • Can be life threatening lymphoma) – High cardiac output state D. Flare of psoriasis due to prednisone taper – Electrolyte imbalance E. Staphylococcal Scalded Skin Syndrome – Respiratory distress – Temperature dysregulation 43 44 11

7/13/16 Psoriasis Psoriasis Comorbidities • 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 • Medications – Systemic steroids – Beta blockers – Lithium – Hydroxychloroquine • Strep infections (children, guttate psoriasis) • Trauma (friction, sunburn) • HIV 47 48 12