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erythropoietic protoporphyria |
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protoporphyria /pro·to·por·phyr·ia/ (prōt″o-por-fēr´e-ah) erythropoietic p. erythropoietic protoporphyria (EPP) a form of erythropoietic porphyria marked by excessive protoporphyrin in erythrocytes, plasma, liver, and feces, and by widely varying photosensitive skin changes ranging from a burning or pruritic sensation to erythema, plaquelike edema, and wheals.
erythropoietic protoporphyria (EPP), an autosomal-dominant disorder, a form of erythropoietic porphyria, characterized by increased levels of protoporphyrin in the erythrocytes, plasma, liver, and feces and a wide variety of photosensitive skin changes, ranging from a burning or pruritic sensation to erythema, plaquelike edema, and wheals. erythropoietic emanating from or pertaining to erythropoiesis. erythropoietic porphyria porphyria of genetic origin; a manifestation of involvement of erythropoietic tissue. erythropoietic protoporphyria protoporphyria of genetic origin in which the defect in porphyrin metabolism is in the erythropoietic tissue. In the bovine disease the deficiency is of heme synthetase (ferrochelatase). How to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit webmaster's page for free fun content. |
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Erythropoietic protoporphyria patients can develop cholestasis, severe hepatic damage, fibrosis, and cirrhosis. |
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