Skin Signs of Systemic Disease
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