Erythropoietic Protoporphyria — Diagnosis and Treatment

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Abstract
Erythropoietic protoporphyria is a disease of porphyrin metabolism characterized by abnormally elevated levels of protoporphyrin IX in erythrocytes, feces and plasma, and by sensitivity to visible light (380 to 560 nm). This sensitivity manifests itself as a burning sensation in the skin, followed by varying degrees of erythema and edema. In consequence, patients with the disorder commonly avoid exposure of the skin to strong light — they tend to choose indoor occupations, nocturnal work, or to venture outdoors while heavily clothed to protect the skin.1 2 3 Symptoms and SignsThe disease is diagnosed in patients with photosensitivity by detection, by chemical . . .

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