Hong Kong Dermatology & Venereology Bulletin

Volume 9 Number 4, December 2001
Abstract

Porphyria Cutanea Tarda and Systemic Diseases - a Report of 10 Cases and Review

Dr. W. K. Fung
Social Hygiene Service (Dermatology), Department of Health, Hong Kong

Porphyria cutanea tarda (PCT) is a metabolic photodermatosis and has been reported in association with a variety of systemic disorders in the West. The clinical characteristics of 10 patients diagnosed to have PCT seen during a 15-year period in four major Dermatology Clinics in Hong Kong were reviewed retrospectively. All 10 patients were type I (sporadic) PCT. Nine patients (90%) were associated with medical diseases. There were five patients with liver-related diseases including hepatitis B, hepatitis C infection and alcoholic cirrhosis; two patients with haematological malignancies including histiocytic lymphoma and myelofibrosis; one patient with systemic lupus erythematosus and one with pulmonary melioidosis. Despite its primarily cutaneous presentation, PCT is often associated with different potentially life-threatening systemic diseases. A review of the literature and evidence supporting a causal association were presented.

Keywords : Porphyria cutanea tarda, review, systemic diseases



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