Porphyria cutanea tarda

Porphyria cutanea tarda (PCT) is the most frequent form of porphyria. The u nderlying enzymatic defect in PCT is a reduced activity of the enzyme uropo rphyrinogen decarboxylase (Uro-D), Four different types of Uro-D disturbanc es are known, Pseudoporphyrias such as porphyria cutanea uraemica or drug-i nduced PCT-like skin symptoms are distinguished from PCT, Porphyrinogens su ch as estrogens or alcohol, or other inducers of P450 isoenzymes provoke PC T, Polymorphisms of P450 isoenzymes, iron overload and infection with hepat itis C virus play an important role in the etiopathogenesis of disease mani festation, Dominant clinical symptoms are bullae, increased cutaneous vulne rability, hypertrichosis and elastosis, Biochemically, total porphyrin leve ls in urine are increased with a predominance of uroporphyrin and heptacarb oxylic porphyrin, Isocoproporphyrin is demonstrable in feces. Best therapeu tic strategies are the oral administration of chloroquine 125 mg twice a we ek and repetitive bloodlettings or the combination of both.