[Erythropoietic protoporthyria. Clinical aspects and therapy]

Abstract

After introduction in clinical, histological and ultrastructural features of erythropoietic protoporphyria the therapeutic effect of beta-Carotene is reported in 5 patients with erythropoietic proporphyria and in 1 patient with polymorphous light dermatosis. beta-Carotene was given orally in a daily initial dose of 50-100 mg, continued with 25 mg a day. In all patients the therapeutic effect set on about 2 months after beginning of the treatment and was measured by increased toleration to insolation. During the treatment over a period of four years no side effects were noticed by 127 clinical and biochemical controls beside a dose-dependent "icterus" localized as well in the perioral region as on the hands and feet.