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April 1974

Nomenclature of Erythropoietic Protoporphyria

Arch Dermatol. 1974;109(4):574-575. doi:10.1001/archderm.1974.01630040078032

To the Editor.—  The metabolic disorder characterized by elevated protoporphyrin in red blood cells (RBC) and feces was first described by Kosenow and Treibs1 in 1953, but it was not clearly defined until the report by Magnus et al2 in 1961. The latter authors named the disease erythropoietic protoporphyria (EPP) because of the obvious involvement of the erythrocytes and normoblasts. Recently, some investigators have concluded that the liver synthesizes most of the excess fecal protoporphyrin, and therefore the disease should be renamed erythrohepatic protoporphyria.3-5 However, as noted by Tschudy et al,6 a systematic classification of the porphyrias will not be possible until all the enzymic defects of these ailments are determined. Because the present level of knowledge is far below this goal, we suggest it would be premature to rename EPP, since EPP is a phrase that is well entrenched in the minds of students of

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