Barbieri L, Griso D, Sorge F, Usai G, Macri A, Biolcati G
Porphyrias Center, S. Gallicano Institute, IRCCS, Rome, Italy
Porphyria cutanea tarda (PCT) is the most frequent cutaneous disorder of porphyrin metabolism and is due to an impaired activity of uroporphyrinogen decarboxylase enzyme. Three major forms of PCT can be distinguished: a sporadic form (acquired, type I) where the erythrocytes enzyme activity is normal but the liver enzyme activity is decreased; an hereditary form (type II) where both erythrocyte and liver enzyme activities are decreased; a familial form (type III) where erythrocyte enzyme activity is normal but the liver enzyme activity is decreased. Hepatitis C virus infection, alcohol and drugs, as estrogens, proved to be some of the most important triggering factors for acquired PCT. We report a case of a 64 years old female patient affected by chronic myeloid leukaemia since 1987. A few months after beginning therapy with antineoplastic agents (hydroxyurea and imatinib esylate) the patient showed skin hyperpigmentation and fragility, hypertrichosis of the temporal regions and blistering of the arms, hands and legs. A clinical suspicion of acquired PCT was made and confirmed by biochemical analysis. Dosage of antineoplastic agents was modified in relation to the course of chronic myeloid leukaemia. We checked porphyrins metabolism during different stages of therapy proving a direct correlation between dosage of antineoplastic drugs and total urine and serum porphyrins values. The authors show the possible porphyrinogenic effect of both the drugs.