Cutaneous Manifestations of Systemic Diseases

Cutaneous Manifestations of Systemic Diseases

Cutaneous Manifestations of Systemic Diseases

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CTDs & Vasculitides - Immune Skin Wars

  • Systemic Lupus Erythematosus (SLE)
    • Malar (butterfly) rash: Spares nasolabial folds.
    • Discoid rash: Erythematous, scaling plaques, follicular plugging, atrophy.
    • Photosensitivity, oral ulcers, alopecia.
    • Raynaud's phenomenon.
  • Dermatomyositis
    • Heliotrope rash: Violaceous periorbital edema.
    • Gottron's papules: Violaceous papules on extensor surfaces of joints (MCP, PIP, DIP).
    • Shawl sign, V-sign: Poikiloderma on sun-exposed areas.
    • Mechanic's hands: Hyperkeratosis, fissuring of palms/fingers.
  • Scleroderma (Systemic Sclerosis)
    • Limited (CREST): Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasias.
    • Diffuse: Widespread skin thickening, early visceral involvement.
    • Salt-and-pepper hypopigmentation/hyperpigmentation.
  • Vasculitides
    • Palpable purpura: Non-blanching, raised lesions (esp. lower limbs).
    • Livedo reticularis, ulcers, digital necrosis.
    • Henoch-Schönlein Purpura (IgA vasculitis): Palpable purpura, arthritis, abdominal pain, renal involvement.

Palpable purpura and bullae in leukocytoclastic vasculitis

Anti-Jo-1 antibodies are strongly associated with interstitial lung disease in dermatomyositis, often presenting with mechanic's hands.

📌 CREST for limited scleroderma features helps recall key manifestations immediately!

Endocrine & Metabolic - Sugar, Spice & Skin Vice

  • Diabetes Mellitus (DM):
    • Necrobiosis lipoidica diabeticorum (NLD): Pretibial, waxy, yellow-brown plaques.
    • Diabetic dermopathy: Atrophic hyperpigmented "shin spots".
    • Acanthosis nigricans: Velvety hyperpigmentation (axillae, neck); sign of insulin resistance.
    • Eruptive xanthomas: Sudden yellow papules (↑ triglycerides).
  • Thyroid Disorders:
    • Hypothyroidism: Myxedema (non-pitting), dry coarse skin, madarosis (📌 Hertoghe's sign: lateral eyebrow loss).
    • Hyperthyroidism (Graves'): Pretibial myxedema (indurated plaques, peau d'orange), thyroid acropachy.
  • Porphyria Cutanea Tarda (PCT):
    • Most common porphyria; photosensitivity, vesicles/bullae, skin fragility, facial hypertrichosis.

    ⭐ PCT is frequently triggered by alcohol, estrogens, Hepatitis C, or hemochromatosis.

  • Xanthomas (Lipid Disorders):
    • Eruptive (↑TG), Tuberous & Tendinous (↑Cholesterol, e.g., Achilles).
    • Xanthelasma palpebrarum: Eyelid plaques.
  • Cushing's Syndrome: Purple striae, skin atrophy, moon facies, buffalo hump.
  • Addison's Disease: Diffuse hyperpigmentation (sun-exposed, buccal mucosa, palmar creases).

Paraneoplastic Syndromes - Cancer's Skin Clues

  • Acanthosis Nigricans (Malignant type): Velvety, hyperpigmented plaques, often in flexures. Assoc: GI adenocarcinoma (esp. stomach).
  • Dermatomyositis: Heliotrope rash, Gottron's papules. Assoc: Ovarian, lung, GI cancers.
  • Leser-Trélat Sign: Sudden onset/↑ in seborrheic keratoses. Assoc: GI (stomach, colon), lymphoma.
  • Necrolytic Migratory Erythema: Annular, erosive lesions. Assoc: Glucagonoma.
  • Sweet's Syndrome (Acute Febrile Neutrophilic Dermatosis): Tender, erythematous plaques/nodules, fever. Assoc: AML.
  • Erythema Gyratum Repens:

GI & Renal Issues - Gut & Kidney Skin News

  • Inflammatory Bowel Disease (IBD):
    • Pyoderma gangrenosum (painful ulcers, violaceous undermined borders)
    • Erythema nodosum (tender red nodules, typically on shins)
    • Oral aphthous ulcers
  • Celiac Disease:
    • Dermatitis herpetiformis (intensely pruritic vesicles on extensor surfaces; IgA deposits)
  • Peutz-Jeghers Syndrome:
    • Mucocutaneous lentigines (lips, oral mucosa, digits)
  • Chronic Kidney Disease (CKD):
    • Uremic pruritus (most common symptom)
    • Xerosis, Calciphylaxis (painful necrosis)
    • Nephrogenic Systemic Fibrosis (post-gadolinium)
    • Half-and-half nails (Lindsay's nails)
  • Acrodermatitis Enteropathica:
    • Zinc deficiency; periorificial & acral dermatitis, alopecia. 📌 ADE = Acral, Diarrhea, Eczema (periorificial).

Cutaneous Manifestations of GI and Renal Diseases

⭐ Dermatitis herpetiformis is strongly associated with celiac disease; skin biopsy shows granular IgA deposits at the dermal-epidermal junction.

High‑Yield Points - ⚡ Biggest Takeaways

  • Acanthosis nigricans: Velvety plaques; linked to insulin resistance (diabetes) & GI malignancy.
  • Dermatomyositis: Heliotrope rash, Gottron's papules; signals underlying malignancy.
  • Erythema nodosum: Tender shin nodules; seen with infections (TB), sarcoidosis, IBD.
  • Pyoderma gangrenosum: Painful ulcers, violaceous borders; associated with IBD, arthritis.
  • Necrobiosis lipoidica: Atrophic shin plaques; classic for diabetes mellitus.
  • Porphyria cutanea tarda: Sun-induced blisters, fragility; linked to liver disease (HCV).
  • SLE: Malar rash, photosensitivity, discoid lesions are key cutaneous signs.

Practice Questions: Cutaneous Manifestations of Systemic Diseases

Test your understanding with these related questions

A 70-year-old man comes to the emergency department because of a skin rash and severe itching. He appears ill; there is a generalized skin rash that is scaly, erythematous, and thickened. His palms, soles, and scalp are also involved. Which of the following is the most likely diagnosis?

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Flashcards: Cutaneous Manifestations of Systemic Diseases

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Acquired ichthyosis is a paraneoplastic syndrome commonly associated with _____

TAP TO REVEAL ANSWER

Acquired ichthyosis is a paraneoplastic syndrome commonly associated with _____

hodgkin's lymphoma

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