Porphyria cutanea tarda: response to vitamin E. A review and two case reports.

Porphyria cutanea tarda is a disease characterized by a triad of cutaneous manifestations: "fragile" skin, usually involving the dorsal aspects of the hands, forearms, legs, or feet; mild hyperpigmentation; and hypertrichosis, especially of the face. The condition is due to a metabolic defect of liver function involving heme synthesis, resulting in the formation of abnormal amounts of uroporphyrin, and sometimes, coproporphyrin or both, which can be measured quantitatively in the urine. Present methods of treatment, including repeated phlebotomy, alkalinization, or chloroquine leave much to be desired. Based on recent experimental and clinical reports and on our personal experience with two patients, we suggest a more logical therapeutic approach in the form of large doses of vitamin E, which apparently corrects the metabolic defect causing the disease.