Advances in the treatment of porphyria cutanea tarda. Effectiveness of slow subcutaneous desferrioxamine infusion.

ABSTRACT

The authors present the results of long-term subcutaneous desferrioxamine (DFX) infusion in 16 porphyria cutanea tarda (PCT) patients who cannot undergo repeated phlebotomies because of severe liver damage, haemolytic anemia, cardiovascular impairment or pulmonary and bone tuberculosis. They employed an automatic, portable syringe pump for subcutaneous infusion (8-10 h) to overcome the short half-life of the drug. Photodynamic cutaneous lesions and hyperpigmentation quickly disappeared (2-3 months). Uroporphyrin excretion sharply decreased and normalized within 3-12 months. Also, serum iron and ferritin, as well as liver function, showed a significant improvement. The authors therefore propose subcutaneous DFX therapy in PCT treatment when phlebotomy is contraindicated.