1/10/2018 Skin Signs of Systemic Disease Dirk Elston MD Professor and Chairman Department of Dermatology and Dermatologic Surgery Medical University of South Carolina 18 y.o. female with persistent sunburn 1 1/10/2018 Acute Cutaneous Lupus • Malar •Pink • Non-scarring • ds-DNA • Penicillamine 2 1/10/2018 Subacute Cutaneous Lupus • Trunk, face, extremities •Pink • Annular or psoriasiform • Ro (SSA), La (SSB) • Neonatal LE • Thiazide diuretics 3 1/10/2018 Discoid Lesions LE panniculitis: Nodular lymphoplasmacytic infiltrates Lobular necrosis Fibrin or hyalin rings Tumid lupus: Periadnexal lymphoid aggregates Dermal mucin 4 1/10/2018 Tumid lupus: Periadnexal lymphoid aggregates Dermal mucin Bywater’s-like Lesions Bywater’s-like Lesions: •Leukocytoclastic vasculitis •Renal and CNS disease 5 1/10/2018 Acral Lupus • Lichenoid clinically • Lichenoid histology • Linear immunoglobulin • SCC in longstanding lesions 6 1/10/2018 Rash on hands 7 1/10/2018 8 1/10/2018 Juvenile DM Image Courtesy of Omar Noor MD 9 1/10/2018 10 1/10/2018 11 1/10/2018 12 1/10/2018 13 1/10/2018 DM vs. LE • Pruritus • Knuckles (vs. phalanges) • Heliotrope •Shawl • Holster sign •Extensors • Nail folds: dilatation and dropout (vs. glomeruloid loops) •45 y.o female with swelling of hands 14 1/10/2018 Acute PSS • Erythema and induration • Usually severe course • Effective treatments for renal disease, reflux • Poor control of skin disease 15 1/10/2018 16 1/10/2018 Morphea profunda: Overlaps histologically with EF Eosinophilic fasciitis: Dry riverbed sign clinically Fascial inflammation, fibrin, sclerosis Infected ear, unresponsive to antibiotics 3rd episode 17 1/10/2018 Relapsing Polychondritis • Clinical diagnosis • H&E: Neutrophils dermal/cartilaginous jct • DIF: Granular band dermal/cart. Jct • Rupture of cardiac valve • Tracheobronchial collapse • Difficult to control Leukocytoclastic vasculitis 18 1/10/2018 Leukocytoclastic vasculitis • Post capillary venule only • No necrosis of endothelium • Little 5 Leukocytoclastic vasculitis • Post capillary venule only • No necrosis of endothelium –HSP – Mixed cryoglobulinemia –Drug – CT disease – Serum sickness 19 1/10/2018 20 1/10/2018 Leukocytoclastic vasculitis • Post capillary venule plus deeper vessels • Necrosis of endothelium •Big 5 Leukocytoclastic vasculitis • Post capillary venule plus deeper • Necrosis of endothelium – Churg-Strauss – Wegener’s – Microscopic polyangiitis – Rheumatoid vasculitis – Septic vasculitis ANCA patterns • Post capillary venule plus deeper • Necrosis of endothelium – Churg-Strauss (p-ANCA) – Wegener’s (c-ANCA) – Microscopic polyangiitis (P-ANCA) – Rheumatoid vasculitis – Septic vasculitis 21 1/10/2018 Retiform purpura • Stellate infacts • Thrombotic: Coagulopathy, levasisole, TTP • Embolic: Cholesterol embolization • Vasculitic: Big 5 Leukocytoclastic vasculitis • Post capillary venule plus deeper • Necrosis of endothelium – Churg-Strauss – Wegener’s – Microscopic polyangiitis – Rheumatoid vasculitis – Septic vasculitis 22 1/10/2018 Janeway Lesion: acute endocarditis Angular purpruic macule with Gun metal gray interior 23 1/10/2018 Meningococcemia 24 1/10/2018 •Post capillary venule only •No necrosis of endothelium Leukocytoclastic vasculitis • Post capillary venule only • No necrosis of endothelium –HSP – Mixed cryoglobulinemia –Drug – Malignancy – CT disease – Serum sickness Drug-induced vasculitis 25 1/10/2018 26 1/10/2018 Churg-Strauss Syndrome 27 1/10/2018 Wegener’s Granulomatosis • Upper airway and renal • c-ANCA • Necrotizing vasculitis • Palisading granulomas with giant cells and PMN’s 28 1/10/2018 29 1/10/2018 Wegener’s Granulomatosis 30 1/10/2018 Calciphylaxis Diabetes Renal failure Rapid weight loss Infection High mortality Treatment: Sodium thiosulfate Tissue plasminogen activator Parathyroidectomy 70 yo woman with warty lips 31 1/10/2018 Paraneoplastic Acanthosis Nigricans • ISH for HPV was negative 32 1/10/2018 Acrodermatitis enteropathica (Zinc deficiency) Diarrhea Dermatitis Failure to thrive 33 1/10/2018 34 1/10/2018 Necrolytic migratory erythema (Glucagonoma syndrome) • Associated with APUD cell tumor of the pancreas, usually in tail of pancreas and usually secretes glucagon • Eruption usually similar to that of zinc deficiency—flexural, acral, periorificial plaques with vesicles and pustules, erosion, crustin • Border is gyrate 35 1/10/2018 Necrolytic acral erythema: Hepatitis C 36 1/10/2018 37 1/10/2018 Porphyria • PCT, VP, HCP: Blisters/milia/hypertrichosis • EPP: Sun hurts, thick red skin • EP: Severe blisters and scarring, red teeth • AIP: No skin signs EPP • Empty Pee Pee Enzymes • EP: Uroporphyrin III cosynthetase • EPP: Ferrochelatase • PCT/HEP: Uro decarboxylase • AIP: Uroporphyrinogen I synthetase • VP: Protoporphyrinogen oxidase • HCP: Coproporphyrin oxidase 38 1/10/2018 39 1/10/2018 Amyloidosis • Primary systemic: skin, mucosa • Secondary systemic: Amyloid rings in fat 40 1/10/2018 41 1/10/2018 Eruptive xanthomas: Diabetes, hypothyroidism Apoprotein C-II: Type I and some Type V Apoprotein E-II: Type III 42 1/10/2018 Tuberous xanthomas 43 1/10/2018 Tendon Xanthomas Palmar Crease Xanthomas (Type III) Apoprotein E2 44 1/10/2018 Normolipemic planar xanthoma: Association with paraproteinemia and myeloma 45 1/10/2018 Langerhans’ Cell Histiocytosis Non-X Histiocytoses: Benign cephalic Generalized eruptive Progressive nodular 46 1/10/2018 Red palms: liver disease, increased estrogen Huntley’s papules: Diabetes mellitus 47 1/10/2018 Yellow nail syndrome: Bronchiectasis Pulmonary effusion Tumors peripheral lung fields Diabetic with new sores on leg Calciphylaxis: Diabetes mellitus, end stage renal disease High mortality 48 1/10/2018 Malignant down (hypertrichosis lanuginosa) 49 1/10/2018 Erythema gyratum repens Paraneoplastic pemphigus Acral Keratoses 50 1/10/2018 Cowden’s Syndrome: Trichilemmomas, sclerotic fibromas, goiter, polyps, breast cancer 51 1/10/2018 Sebaceous adenoma 52 1/10/2018 Muir-Torre: Sebaceous neoplasms, Keratoacanthomas, Gut carcinoma Acanthosis nigricans: Endocrine, syndromal, Malignancy (insulin) Necrolytic migratory Erythema: glucagonoma 53 1/10/2018 Multiple mucosal neuromas: MEN IIb NF1 and JXG: Association with CML 54.