Özet:

Porphyria cutaneatarda (PCT) is a rare disease of porphyrin metabolism, related to a deficiency of Uroporphyrinogen decarboxylase activity. Clinically, it manifests itself by a skin fragility in photo-exposed areas. The characteristic biochemical profile of PCT, with elevated levels of urinary and plasma porphyrins, establishes the diagnosis. Treatment is based on phlebotomy, hydroxychloroquine (100 to 200 mg twice weekly) and control of susceptibility factors. We report a case of porphyria cutaneatarda in a male coast guard by profession.

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