vs_derm.ppt333

Cutaneous Manifestations of Systemic Disease

Holly Edmonds, MD Chief Resident Department of Dermatology

Brief Review of terminology…

Zits???

Red Rash???

“Nasty… just call the derm service and get a consult!”

You have to learn the language!

Description of a skin lesion:
Type Shape Arrangment Distribution OH!!! DATS what that rash is!!!

TYPE

Primary lesion Secondary lesion Color Palpation

MACULE/PATCH

PLAQUE

PAPULE

NODULE

VESICLE/BULLA

PUSTULE

WHEAL

SCALE

CRUST

LICHENIFICATION

EROSION

ULCER

ATROPHY

EXCORIATION

FISSURE

SCAR

Color

Color

Color

Palpation

Consistency Firm, Soft, Fluctuant, Boardlike Temperature deviation (hot or cold) Mobility Presence of tenderness

Margination

Ill-defined vs. Well-defined

SHAPE

SHAPE

SHAPE

SHAPE

SHAPE

ARRANGEMENT

ARRANGEMENT

ARRANGEMENT

ARRANGEMENT

ARRANGEMENT

DISTRIBUTION

DISTRIBUTION

DISTRIBUTION

DISTRIBUTION

DISTRIBUTION

Cutaneous Manifestations

Diabetes Mellitus Renal Disease Gastrointestinal disorders Rheumatologic disease Hepatitis C Thyroid disease Paraneoplastic disease Nutritional disease

Diabetes Mellitus

Acanthosis Nigricans Diabetic Dermopathy Bullosis Diabeticorum Necrobiosis Lipoidica Diabetic Foot Ulcers

Acanthosis Nigricans

African Americans and Hispanics > Caucasians Associated with obesity, insulin resistance Hyperpigmented velvety plaques of the flexures Genetic sensitivity of the skin to hyperinsulinemia Malignant form a/w gastric ACA

Acanthosis Nigricans

Acanthosis Nigricans

Diabetic Dermopathy

AKA “shin spots” or pigmented pretibial papulesMost common cutaneous manifestation of diabetesBenign asymptomatic red brown macules on shinsNo treatment needed

Diabetic Dermopathy

Bullosis Diabeticorum
Rapid onset painless, tense blisters on hands and feet Trauma and microvascular disease may play a role Spontaneous healing in 2-5 weeks

Bullosis Diabeticorum

Necrobiosis Lipoidica
20-35% of patients with NLD have diabetes, only about 1-3% of diabetics have NLD Start as red-brown papules and progress to well defined yellow-brown atrophic plaques with irregular violaceous borders and telangiectasias. Shins #1 site. Ulceration in 35%. Glucose control will not clear NLD

Necrobiosis Lipoidica Diabeticorum

Diabetic Eruptive Xanthomas

Seen in uncontrolled diabetes, hypertriglyceridemia Sudden crops on firm, non-tender yellow papules with a red rim on extensors Control glucose and lipid reduction will reduce lesions

Diabetic Eruptive Xanthomas

Diabetic Neurotropic Ulcers
Peripheral neuropathy leads to unnoticed trauma Vascular complications may lead to ulcers and complicate ulcer healing Risk of amputation goes up 8x once these develop

Diabetic Neurotropic Ulcers

Renal disease
Pruritis Perforating dermatosis Calciphylaxis Nephrogenic Fibrosing Dermopathy

Pruritis

Most common cutaneous manifestation of renal disease Seen in both peritoneal and hemodialysis patients Unknown mechanism, unsatisfactory therapy- UVB helps the most

Pruritis

Perforating Disorder

Acquired Perforating Dermatosis of ESRD Umbilicated papules/nodules with central hyperkeratotic core

Perforating dermatosis

Perforating dermatosis

Calciphylaxis

Painful purpuric plaques and retiform purpura More proximal lesions = poorer prognosis

Calciphylaxis

Nephrogenic Fibrosing Dermopathy
Woody, indurated plaques with peau d’orange appearanceUsually spares the face, palms, solesAssociated with gadolinium contrast for MRIs


Nephrogenic Fibrosing Dermopathy

Gastrointestinal disorders

Henoch Schonlein Purpura Dermatitis Herpetiformis Inflammatory Bowel Disease

Henoch Schonlein Purpura

Palpable purpura, urticaria, necrotic ulcers on buttocks, distal legs Symmetric IgA vasculitis GI symptoms + arthritis, long term concern for hypertension and renal involvement Usually under 20, following an URI


HSP


HSP

Dermatitis Herpetiformis

Symmetric, grouped vesicles on extensorsVery pruritic!All patients have gluten-sensitive enteropathy, only 20% symptomaticAssociated with HLA-DQ2, Hashiomoto’s thyroiditis, lymphoma, IDDMCutaneous findings are due to autoantibodies to epidermal transglutaminaseTreatment- rapidly responsive to dapsone

Dermatitis Herpetiformis

Inflammatory Bowel Disease

Crohn’s and Ulcerative Colitis

Oral Crohn’s Linear ulcerations, cobblestoned oral mucosa UC may have aphthous ulcers that develop as IBD flares

Metastatic Crohn’s See fissures and fistulas with Crohn’sMetastatic Crohn’s are nodules, plaques and ulcerations usually in intertriginous areas which can mimic erythema nodosum

Erythema Nodosum

Tender red nodules on anterior lower legs, precedes or occurs with IBD flares, UC more common Most EN is idiopathic, also can be related to oral contraceptives or abx, preceding strep or mycobacterial infxn

Pyoderma Gangrenosum

More common in UC Papules, pustules, hemorrhagic blisters enlarge and ulcerate with dusky undermined edges Frequently on legs or around stoma sites Treat with steroids, often gets better as IBD gets better

Rheumatologic Disorders

Lupus ErythematosisDermatomyositisReiter’s Disease

Lupus Erythematosis

Classification: Systemic Cutaneous Lupus (SLE) Subacute Cutaneous Lupus (SCLE) Discoid Lupus (DLE) Neonatal Lupus



SLE
+ANA +Sm and dsDNA Butterfly Rash Poikiloderma Photodistrubited erythematous, papular scaling eruption sparing knuckles.

SLE


SLE


SLE

Dermatomyositis

Poikiloderma favoring scalp, periocular (Heliotrope rash), and extensor skin sites Nailfold telangiectasiasGottron’s papules

Dermatomyositis

Dermatomyositis(periungal telangiectasias, gottrons papules)



Reiter’s Disease Urethritis, arthritis, ocular findings, and oral ulcers in addition to psoriasiform skin lesions. Keratoderma blenorrhagicum (feet) Balanitis circinata (penis)

Reiter’s DiseaseKeratoderma Blenorrhagicum

Reiter’s Disease(balanitis circinata)

Hepatitis C virus

Porphyria Cutanea Tarda Lichen Planus Pruritis

Porphyria Cutanea Tarda

Vesicles and bullae on sun-exposed areas, scarring with milia Hypertrichosis Fragile skin with sclerodermoid changes


PCT

PCT(hypertrichosis)

Lichen Planus
Purple, pruritic, polygonal papulesKoebner phenomenonWickham’s striae50% with mucosal involvement

Lichen Planus

Mucosal Lichen Planus

Pruritis

Excoriations, lichenification, and prurigo nodularis

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Pruritis

Thyroid disease
Graves disease Hyperthyroidism Hypothyroidism

Graves Disease

Thyroid dermopathy (pretibial myxedema) Symmetric, non-pitting yellow-brown waxy papules/plaques Due to increased hyaluronic acis in dermis

Thyroid Dermopathy

Hyperthyroidism
Warm, moist skin Flushing, palmar erythema Associated with reversible alopecia and vitiligo

Vitiligo

Hypothyroidism
Dry, cool skin Generalized myxedema Yellow hue from carotenemia Purpura from delayed wound healing Alopecia, madarosis

Carotenemia

Paraneoplastic Disorders
Acanthosis NigricansDermatomyositisCushing’s DiseaseSign of Lesser-TrelatParaneoplastic PemphigusHypertrichosis Lanuginosa

Acanthosis Nigricans

Dermatomyositis

Cushing’s syndrome(buffalo hump and striae)

Sign of Lesser-Trelat

Paraneoplastic Pemphigus

Erythema Gyratum Repens(associated with cancers above diaphragm)

Hypertricosis Lanuginosa Acquisita

Nutritional Disorders

1. Marasmus 2. Kwashiorkor 3. Pellagra 4. Scurvy 5. Zinc deficiency

Marasmus

Marasmus = protein/calorie malnutrition. Cutaneous Manifestations: Emaciation with thin, lax, and wrinkled skin. Fine scaling with hyperpigmentation. Follicular hyperkeratosis Purpura Thin hair and nails.

Marasmus

Marasmus

Kwashiorkor

Decreased protein intake Dyschromia (irregular pigment) Hypopigmentation. Superficial desquamation with areas of erosion (flaky paint) Petechia/purpura Thin hair/nails

Kwashiorkor

Kwashiorkor

Pellagra

Niacin deficiency (Vit B3)Triad of dermatitis, diarrhea, dementiaPhotosensitive eruption around neck known as “Casal’s necklace”

Pellagra

PellagraCasal’s neckace

Scurvy

Vitamin C deficiency Follicular hyperkeratosis with corkscrew hairs Perifollicular hemorrhage Gingival hypertrophy with erosive bleeding gums.

Scurvy

Zinc Deficiency
Acquired -deficient intake, high fiber intake, malabsorption Inherited (acrodermatitis enteropathica) zinc deficiency -defect in intestinal absorption of zinc Dermatitis, diarrhea, alopecia Periorificial and acral distribution

Acrodermatitis Enteropathica

Acrodermatitis Enteropathica

A couple of randoms you should know…

Sarcoidosis
Multisystem granulomatous disease Skin affected in 20-35%

Neurofibromatosis

Von Reckinghausen’s diseaseAutosomal dominantNeurofibromin gene, Chr 17

Tuberous Sclerosis

Autosomal Dominant Hamartin and Tuberin (TSC 1 and 2) MR, Seizures (variable)

QUIZ TIME!!!

Very itchy.

Lichen Planus (HCV)

Anterior lower leg

Necrobiosis Lipoidica (DM)

?

Pyoderma Gangrenosum (IBD)

?

Porphyria Cutanea Tarda

?


SLE


?

Basal Cell Carcinoma

Nodulocystic Acne

Xanthelasma

Herpes Labialis

Nevus Sebaceous