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Nomenclature of Erythropoietic Protoporphyria

Nomenclature of Erythropoietic Protoporphyria Abstract 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 References 1. Kosenow W, Treibs A: Lichtüberempfindlichkeit und Porphyrinamie . Z Kinderheilkd 73:82-92, 1953.Crossref 2. Magnus IA, et al: Erythropoietic protoporphyria, a new porphyria syndrome with solar uticaria due to protoporphyrinaemia . Lancet 2:448-451, 1961.Crossref 3. Scholnick P, Marver HS, Schmid R: Erythropoietic protoporphyria: Evidence for multiple sites of excess protoporphyrin formation . J Clin Invest 50:203-207, 1971.Crossref 4. Elder GH, Gray CH, Nicholson DC: The porphyrias: A review . J Clin Pathol 25:1013-1033, 1972.Crossref 5. Nicholson, et al: Isotope studies of the erythropoietic and hepatic components of congenital porphyria and `erythropoietic' protoporphyria . Clin Sci 44:135-150, 1973. 6. Tschudy DP, Magnus IA, Kalivas J: The porphyrias , in Fitzpatrick TB, et al (eds): Dermatology in General Medicine . New York, McGraw-Hill Book Co Inc, 1971, p 1147. 7. Schwartz S, et al: Erythropoietic defects in protoporphyria: A study of factors involved in labelling of porphyrins and bile pigments from ALA-3H and glycine-14C . J Lab Clin Med 78:411-434, 1971. 8. Gray CH, et al: Isotope studies on a case of erythropoietic protoporphyria . Clin Sci 26:7-15, 1964. 9. Cripps DJ, MacEachern WN: Hepatic and erythropoietic protoporphyria . Arch Pathol 91:497-505, 1971. 10. Marver HS, Schmid R: The porphyrias , in Stanbury JB, Wyngaarden JB, Fredrickson DS (eds): The Metabolic Basis of Inherited Disease , ed 3. New York, McGraw-Hill Book Co Inc, 1972, p 1088. 11. Kaplowitz N, Javitt N, Harber LC: Isolation of erythrocytes with normal protoporphyrin levels in erythropoietic protoporphyria . N Engl J Med 278:1077-1081, 1968.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Nomenclature of Erythropoietic Protoporphyria

Archives of Dermatology , Volume 109 (4) – Apr 1, 1974

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Publisher
American Medical Association
Copyright
Copyright © 1974 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1974.01630040078032
Publisher site
See Article on Publisher Site

Abstract

Abstract 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 References 1. Kosenow W, Treibs A: Lichtüberempfindlichkeit und Porphyrinamie . Z Kinderheilkd 73:82-92, 1953.Crossref 2. Magnus IA, et al: Erythropoietic protoporphyria, a new porphyria syndrome with solar uticaria due to protoporphyrinaemia . Lancet 2:448-451, 1961.Crossref 3. Scholnick P, Marver HS, Schmid R: Erythropoietic protoporphyria: Evidence for multiple sites of excess protoporphyrin formation . J Clin Invest 50:203-207, 1971.Crossref 4. Elder GH, Gray CH, Nicholson DC: The porphyrias: A review . J Clin Pathol 25:1013-1033, 1972.Crossref 5. Nicholson, et al: Isotope studies of the erythropoietic and hepatic components of congenital porphyria and `erythropoietic' protoporphyria . Clin Sci 44:135-150, 1973. 6. Tschudy DP, Magnus IA, Kalivas J: The porphyrias , in Fitzpatrick TB, et al (eds): Dermatology in General Medicine . New York, McGraw-Hill Book Co Inc, 1971, p 1147. 7. Schwartz S, et al: Erythropoietic defects in protoporphyria: A study of factors involved in labelling of porphyrins and bile pigments from ALA-3H and glycine-14C . J Lab Clin Med 78:411-434, 1971. 8. Gray CH, et al: Isotope studies on a case of erythropoietic protoporphyria . Clin Sci 26:7-15, 1964. 9. Cripps DJ, MacEachern WN: Hepatic and erythropoietic protoporphyria . Arch Pathol 91:497-505, 1971. 10. Marver HS, Schmid R: The porphyrias , in Stanbury JB, Wyngaarden JB, Fredrickson DS (eds): The Metabolic Basis of Inherited Disease , ed 3. New York, McGraw-Hill Book Co Inc, 1972, p 1088. 11. Kaplowitz N, Javitt N, Harber LC: Isolation of erythrocytes with normal protoporphyrin levels in erythropoietic protoporphyria . N Engl J Med 278:1077-1081, 1968.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Apr 1, 1974

References