Therapy of porphyria cutanea tarda.
Porphyria cutanea tarda (PCT) is the most common type of porphyria. There is an association of PCT with haemochromatosis, diabetes mellitus and hepatitis C infection. The basis of treatment of PCT consists of three elements: avoidance of triggering factors, iron depletion and porphyrin elimination. Alcohol and certain systemic medical drugs, such as oestrogens (or tuberculostatics), should be considered as triggering factors, and as far as possible, avoided. Other triggering factors, such as chronic haemodialysis in renal insufficiency, need a different approach. The hallmark in iron depletion is phlebotomy. Porphyrin elimination is achieved using low-dose chloroquin therapy. The treatment is safe and effective but has its limits in cases with haemochromatosis (HFE) gene mutations. Here iron depletion needs additional phlebotomy. In patients with chronic haemodialysis-associated PCT, chloroquine is ineffective. Erythropoietin, desferroxamine and small-volume phlebotomy have been employed to control the disease. Childhood PCT is very rare. No controlled studies are available, but published experience suggests that body weight-adapted chloroquine therapy or small volume phlebotomy might be useful.
Köstler
Erich
E
Academic Teaching Hospital, Department of Dermatology, Dresden-Friedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany. koestler-er@khdf.de
Wollina
Uwe
U
D002738:Chloroquine / Q000627:therapeutic use; D003676:Deferoxamine / Q000627:therapeutic use; D004921:Erythropoietin / Q000627:therapeutic use; D006801:Humans; D007501:Iron / Q000378:metabolism; D018962:Phlebotomy; D017119:Porphyria Cutanea Tarda / Q000188:drug therapy* / Q000209:etiology; D006435:Renal Dialysis / Q000009:adverse effects; D013473:Sunscreening Agents
D013473:Sunscreening Agents; D004921:Erythropoietin; D002738:Chloroquine; D007501:Iron; D003676:Deferoxamine