Porphyria cutanea tarda

Abstract

A 68-year-old man with relapsed follicular lymphoma developed redness, fragility, recurrent painful blistering and ulcerating lesions on sun-exposed areas of the skin, especially on the back of the hands (Figures). The appearance of the lesions led us to suspect porphyria cutanea tarda (PCT) which was confirmed by elevated urinary excretion of uroporphyrin, hepta-, hexaand pentacarboxylic porphyrins, as well as coproporphyrin. PCT is the most common type of porphyric disorder, triggered by hepatic uroporphyrinogen decarboxylase deficiency. Clinical manifestations include hypertrichosis and a photosensitive bullous dermatosis, predominantly on the hands, forearms, back of the neck and face. PCT may be caused by hepatic siderosis, alcohol abuse, hepatitis C virus infection or oestrogen therapy. During the course of his disease the patient had received several blood transfusions and his serum ferritin concentration was increased (serum ferritin: 2410 lg/l, normal range: 20–290 lg/l). The patient was treated with chloroquine to facilitate urinary elimination of porphyrins and the underlying lymphoma was treated with chemotherapy. Six months later the skin lesions had completely resolved in parallel with lymphoma remission and markedly decreased ferritin levels (serum ferritin: 1130 lg/l).