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
John A. Johnson, Ramon M. Fusaro. Nomenclature of Erythropoietic Protoporphyria. Arch Dermatol. 1974;109(4):574–575. doi:10.1001/archderm.1974.01630040078032
* * Maintenance Notice * *
Maintenance will be performed from 11:00pm CT December 16, 2017 to 4:00pm CT December 17. Please be advised that your site/services may be periodically unavailable during this maintenance window.