Frequency of Occurrence of Hepatocellular Carcinoma in Patients With Porphyria Cutanea Tarda in Long-term Follow-up

Abstract

Porphyria cutanea tarda (PCT) occupies a significant place among porphyrias by generally being considered an acquired disease, even though certain genetic influences cannot be wholly excluded. Some writers agree that in this type of porphyria the liver is very often affected by fibrosis of varying degrees, which in the great majority of cases develops into cirrhosis (1, 2, 3). Nevertheless, the pathogenesis of these hepatic alterations has not yet been satisfactorily explained. Available reports on autopsy findings of PCT are few, and likewise long-term follow-ups of the development of this disease in a broader clinical context are lacking. To study the problem in connection with injury to the liver parenchyma in patients with PCT, we utilized material of the porphyric unit of the First Medical Department, Faculty of General Medicine, Prague.