A light and electron microscopic study of the liver in case of erythrohepatic protoporphyria and in griseofulvin induced porphyria in mice

Abstract

The porphyrias may be classified as 'hepatic' or 'erythropoietic' according to whether the liver or bone marrow is the principal site of disturbed porphyrin metabolism. Usually included in the erythropoietic group is the inherited disorder erythropoietic protoporphyria (EPP). The site of the principal lesion is not yet known but present evidence suggests the existence of a hepatic as well as an erythropoietic lesion. Following reports of intrahepatic deposits of protoporphyrin in erythropoietic protoporphyria, cases of cirrhosis and liver cell failure have been described and the term 'erythrohepatic protoporphyria has been suggested as a more comprehensive description of the disease. A disturbance of porphyrin metabolism characterized by biochemical changes similar to those of human erythropoietic protoporphyria and the deposition of protoporphyrin in liver cells follows the administration of griseofulvin to mice. A study was made of the light microscopic and ultrastructural appearances of a liver biopsy from a patient with erythropoietic protoporphyria who had abnormal liver function tests. The results were compared with the morphologic changes in the livers of mice during the development of griseofulvin induced porphyria.