Liver & intrahepatic bile ducts
Metabolic diseases
Erythropoietic protoporphyria
Author: Komal Arora, M.D.
Last author update: 1 April 2012
Last staff update: 29 January 2024
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Erythropoietic protoporphyria liver
Table of Contents
Definition / general | Diagrams / tables | Gross images | Microscopic (histologic) description | Electron microscopy descriptionCite this page: Arora K. Erythropoietic protoporphyria. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livererythropoieticprotoporphyria.html. Accessed December 22nd, 2024.
Definition / general
- Excess production of free protoporphyrin from the bone marrow, most commonly due to deficiency of the enzyme ferrochelatase; causes characteristic cutaneous photosensitivity and in a minority of patients leads to end stage liver disease necessitating liver transplantation (J Inherit Metab Dis 2011;34:539, World J Gastroenterol 2010;16:4526)
- Paracrystalin protoporphyrin deposition in hepatocytes and precipitation in the biliary canaliculi
- Prognosis depends on extent of liver disease (Orphanet J Rare Dis 2009;4:19)
Diagrams / tables
Images hosted on other servers:
Heme biosynthetic pathway
Gross images
Images hosted on other servers:
Explanted liver
Microscopic (histologic) description
- Deep brown colored bile in canaliculi, bile ducts and Kupffer cells with red Maltese cross under polarized light
Electron microscopy description
- Starburst crystalline array
