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April 1980

The Consequences of Not Diagnosing Erythropoietic Protoporphyria

Arch Dermatol. 1980;116(4):407. doi:10.1001/archderm.1980.01640280043014

Erythropoietic protoporphyria (EPP) is usually considered a mild disease, with its manifestations primarily limited to photosensitivity of the skin, though some increased incidence of gallbladder disease has been noted.1,2 However, within the last decade, the occurrence of 15 cases of fatal liver disease in patients with EPP sugests that in at least some patients, EPP is not a benign condition.3 With the 16th report of a fatal case by Wells et al in this issue (see p 429), two points need to be emphasized: (1)because of the possibility that life-threatening liver disease, which could be reversed in its early stages, may develop in some patients with EPP it is imperative to test for the presence of elevated erythrocyte, plasma, or stool porphyrin levels in every case of photosensitivity to visible light, and (2) because of the possibility of the development of liver disease, all patients with EPP should

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