Cutaneous Manifestations of Systemic Disease

Cutaneous Manifestations of Systemic Disease

Cutaneous Manifestations of Systemic Disease

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Autoimmune Manifestations - Immune System Alerts

  • Systemic Lupus Erythematosus (SLE)
    • Malar (butterfly) rash, discoid lesions, photosensitivity.
    • Oral ulcers, non-scarring alopecia, Raynaud's. ANA often positive. Malar rash in systemic lupus erythematosus
  • Dermatomyositis
    • Heliotrope rash (eyelids), Gottron's papules (knuckles).
    • Shawl sign, V-sign, mechanic's hands. Periungual telangiectasias.
    • Associated with internal malignancy (adults > 40 yrs). Anti-Jo-1, Anti-Mi-2.
  • Scleroderma (Systemic Sclerosis)
    • Skin thickening/fibrosis (sclerodactyly), Raynaud's (often first).
    • Telangiectasias, calcinosis cutis, "salt-and-pepper" dyspigmentation.
    • 📌 CREST: Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia. Anti-centromere Ab.
  • Pemphigus Vulgaris
    • Flaccid bullae, erosions (oral mucosa often first).
    • Nikolsky sign positive. Intraepidermal acantholysis.

    ⭐ Antibodies against desmoglein 1 & 3 (anti-Dsg1, anti-Dsg3).

  • Bullous Pemphigoid
    • Tense bullae on erythematous/urticarial base (elderly).
    • Nikolsky sign negative. Subepidermal split with eosinophils. Anti-BPAG1/2.

Metabolic & Endocrine - Inside‑Out Clues

  • Diabetes Mellitus:
    • Necrobiosis lipoidica (atrophic plaques, shins)
    • Diabetic dermopathy (shin spots)
    • Acanthosis nigricans (velvety, flexures) Acanthosis nigricans in axilla
    • Eruptive xanthomas (↑ triglycerides)
    • Bullosis diabeticorum
  • Thyroid Disorders:
    • Hypo: Myxedema, dry skin, madarosis (outer eyebrow loss)
    • Hyper (Graves'): Pretibial myxedema, acropachy, Plummer's nails
  • Adrenal Disorders:
    • Cushing's: Violaceous striae (>1 cm), moon facies, buffalo hump, atrophy
    • Addison's: Hyperpigmentation (oral, palmar, scars)
  • Xanthomas:
    • Xanthelasma palpebrarum (eyelids; normolipidemic common)
    • Tuberous, tendinous, eruptive (hyperlipidemia)
  • Porphyria Cutanea Tarda (PCT): Photosensitivity, bullae (sun-exposed), fragile skin, hypertrichosis, port-wine urine.

    ⭐ Acanthosis nigricans can be a sign of insulin resistance (e.g., diabetes) or internal malignancy (especially gastric adenocarcinoma).

GI & Nutritional - Gut‑Skin Signals

  • IBD (Crohn's/UC):
    • Erythema nodosum (EN), Pyoderma gangrenosum (PG).
    • Oral aphthae, cutaneous Crohn's.
  • Celiac Disease:
    • Dermatitis herpetiformis (DH): intensely pruritic vesicles (extensors); IgA at DEJ. Dermatitis herpetiformis vesicles on elbows
    • Angular cheilitis, glossitis.

⭐ Dermatitis herpetiformis is a highly specific cutaneous marker for Celiac Disease, responding to a gluten-free diet.

  • Nutritional Deficiencies:
    • Zinc (Acrodermatitis Enteropathica): Periorificial/acral dermatitis, alopecia, diarrhea.
    • Niacin/B3 (Pellagra): 📌 3 D's: Dermatitis (photosensitive, Casal's necklace), Diarrhea, Dementia.
    • Vit C (Scurvy): Perifollicular hemorrhage, corkscrew hairs, gingivitis.
  • Liver Disease: Pruritus, jaundice, spider nevi, palmar erythema.
  • Pancreatic Disease: Enzymatic panniculitis (painful nodules), Trousseau's sign (migratory thrombophlebitis with malignancy).

Paraneoplastic & Vascular - Cancer & Vessel Alarms

  • Paraneoplastic Syndromes (Skin signals of internal cancer):
    • Acanthosis Nigricans: GI (stomach), lung. Velvety skin (axilla, neck).
    • Leser-Trélat sign: Sudden ↑ seborrheic keratoses. GI (stomach, colon).
    • Tripe Palms: Gastric/Lung cancer. Rugose palms.
    • Dermatomyositis: Gottron's papules, heliotrope rash. Ovarian, lung. 📌 "Got Heli?"
    • Necrolytic Migratory Erythema: Glucagonoma. Annular crusted plaques.
    • Sweet's Syndrome: AML. Tender red plaques.
  • Cutaneous Vasculitis (Vessel inflammation):
    • Palpable purpura: Hallmark. Non-blanching. Lower limbs.
    • Leukocytoclastic Vasculitis (LCV): Small vessels. Drugs, infections, malignancy.
    • IgA Vasculitis (HSP): Purpura, arthritis, abdominal pain, renal.
    • Polyarteritis Nodosa (PAN): Medium vessels. Livedo reticularis, nodules, ulcers. HBV. Leser-Trelat sign: multiple seborrheic keratoses

⭐ Leser-Trélat sign: abrupt onset of multiple seborrheic keratoses, strongly linked to internal malignancy, especially GI adenocarcinoma.

High‑Yield Points - ⚡ Biggest Takeaways

  • Acanthosis nigricans: Velvety plaques; linked to insulin resistance and gastric cancer.
  • Dermatomyositis: Gottron's papules, heliotrope rash; signals underlying malignancy.
  • Erythema nodosum: Tender shin nodules; associated with sarcoidosis, infections, IBD.
  • Pyoderma gangrenosum: Painful ulcers with violaceous borders; linked to IBD.
  • Porphyria cutanea tarda: Sun-exposed blisters; associated with liver disease, HCV.
  • Necrobiosis lipoidica: Atrophic shin plaques; strongly indicates diabetes mellitus.
  • Sweet's syndrome: Tender plaques, fever; consider Acute Myeloid Leukemia (AML).

Practice Questions: Cutaneous Manifestations of Systemic Disease

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A female patient of 26 years, presents with oral ulcers, photosensitivity and skin malar rash in face sparing the nasolabial folds of both side.

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Flashcards: Cutaneous Manifestations of Systemic Disease

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Photoallergic contact dermatitis is a type _____ hypersensitivity reaction

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Photoallergic contact dermatitis is a type _____ hypersensitivity reaction

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