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Therapeutic Approach

Porphyria cutanea tarda (PCT) is the most common porphyria and is characterized by the development of skin friability and chronic, blistering lesions on the dorsal aspects of the hands and other sun-exposed areas of skin usually in mid or late life. PCT is an iron-related disease resulting from inhibition of hepatic uroporphyrinogen decarboxylase (UROD, the 5th enzyme in the heme biosynthetic pathway). This develops most commonly in adult males in association with acquired susceptibility factors such as excess alcohol use, smoking, chronic hepatitis C or HIV infection and, particularly in females, with estrogen use. Genetic susceptibility factors may include heterozygous UROD mutations (such patients are said to have familial PCT) and HFE (hemochromatosis gene) mutations. PCT responds readily to repeated phlebotomy, low-dose hydroxychloroquine or treatment of hepatitis C (if present). Before treatment is initiated, the disease must be differentiated from other less common porphyrias that cause identical skin lesions but are unresponsive to these treatments. See Table 121-1.

Table 121-1Porphyria Cutanea Tarda Treatment Table

Levels of evidence are based on the Journal of the American Academy of ...

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