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September 1974

Congenital Erythropoietic Porphyria: An Autopsy Report

Author Affiliations

From the Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi.

Arch Dermatol. 1974;110(3):427-431. doi:10.1001/archderm.1974.01630090061013

Autopsy findings of a patient who had classical, clinical and biochemical features of congenital erythropoietic porphyria are presented. Under Wood light, all the viscera, particularly the liver, the kidneys, the intestines, and all the bones showed orange-red fluorescence. Microscopy showed fluorescence in the red blood cells, skin, the bile ducts, and the renal glomeruli and tubules. Tuberculoid granulomas were detected in the liver and in the mesenteric lymph nodes, but no microbes could be demonstrated. The granulomatous change probably represents a reaction to abnormal deposits of porphyrins.

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