erythropoietic protoporphyria (redirected from Protoporphyria, erythropoietic)

protoporphyria /pro·to·por·phyr·ia/ (prōt″o-por-fēr´e-ah) erythropoietic p.

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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.

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erythropoietic protoporphyria

n.

A benign inherited disorder of porphyrin metabolism characterized by enhanced fecal excretion of protoporphyrin and elevated concentration of protoporphyrin in red blood cells, plasma, and feces, with acute solar urticaria or chronic solar eczema appearing quickly upon exposure to sunlight.

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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.

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erythropoietic

emanating from or pertaining to erythropoiesis.

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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).