Definition of Porphyria Cutanea Tarda
Porphyria cutanea tarda: Literally, the late skin form of porphyria, a genetic photosensitive (light-sensitive) skin disease with onset in adult life with substances called uroporphyrins in the urine due to a deficiency of uroporphyrinogen decarboxylase (UROD), an enzyme required for the synthesis of heme (part of hemoglobin, the pigment in red blood cells that carries oxygen). The hallmarks of porphyria cutanea tarda (PCT) are blisters which become ulcerated in areas of the skin exposed to sunlight, especially on the face, ears and dorsum (back) of the hands. The affected areas of skin also tend to be fragile and show hyperpigmentation (excess pigment) and hypertrichosis (excess hair).
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