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Key Features

  • Noninflammatory blisters on sun-exposed sites, usually only the dorsal hands

  • Hypertrichosis, skin fragility

  • Associated liver disease

  • Elevated urine porphyrins

  • The disease is associated with ingestion of certain medications (eg, estrogens), and alcoholic liver disease or hepatitis C

Clinical Findings

  • Painless blistering and fragility of the skin of the dorsal surfaces of the hands

  • Facial hypertrichosis and hyperpigmentation

  • In patients with liver disease, hemosiderosis is often present

  • Differential diagnosis

    • Pseudoporphyria: dialysis, medications (tetracycline, nonsteroidal anti-inflammatory drugs, voriconazole)

    • Contact dermatitis

    • Scabies

Diagnosis

  • Urinary uroporphyrins are elevated two- to five-fold above coproporphyrins

  • There may be abnormal liver biochemical tests, evidence of hepatitis C infection, increased liver iron stores, and various hemochromatosis gene mutations

Treatment

  • Phlebotomy without oral iron supplementation at a rate of 1 unit every 2–4 weeks will gradually lead to improvement

  • Hydroxychloroquine 200 mg orally twice weekly will increase the excretion of porphyrins, improving the skin disease

  • Deferasirox, an iron chelator can also improve porphyria cutanea tarda

  • Stopping all triggering medications and stopping alcohol consumption may lead to improvement

  • Most patients improve with treatment

  • Barrier sun protection with clothing is required for prevention

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