PMID: 6989325Apr 1, 1980Paper

Erythropoietic protoporphyria with hepatic cirrhosis

Archives of Dermatology
M M WellsB J Bender

Abstract

Cholestatic jaundice and rapidly deteriorating hepatic function developed in a 19-year-old man with a lifelong history of photosensitivity. Laboratory studies revealed the characteristic increased erythrocyte and fecal protoporphyrin levels of erythropoietic protoporphyria. Progressive hepatic failure was treated by orthotopic liver transplantation six months after the first clinical indication of hepatic dysfunction. Characteristic light microscopic, fluorescence microscopic, and electron microscopic findings of erythropoietic protoporphyria were present in skin and liver. Four weeks after liver transplantation, the patient died of disseminated candidiasis. At autopsy, the donor liver had no microscopic evidence of protoporphyrin accumulation, although tissue protoporphyrin levels were mildly elevated.

Citations

Oct 1, 1983·The Journal of Clinical Investigation·M B Poh-FitzpatrickJ H Lefkowitch
Sep 12, 2009·Orphanet Journal of Rare Diseases·Mario LechaJean-Charles Deybach
Apr 5, 2014·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Ashwani K SingalBrendan M McGuire
Apr 9, 2010·The Korean Journal of Hepatology·Dong Jun YooDong Jin Suh
Nov 30, 2005·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Neville Roy Pimstone
Nov 30, 2005·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Brendan M McGuireJoseph R Bloomer
Jan 21, 2003·The Journal of Pathology·Louis LibbrechtPeter Jansen
May 24, 2011·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Staffan WahlinUNKNOWN European Liver and Intestine Transplant Association

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