Liver and intrahepatic bile ducts - nontumor
Metabolic diseases
Erythropoietic protoporphyria



Topic Completed: 1 April 2012

Revised: 23 January 2019, last major update April 2012

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PubMed Search: Erythropoietic protoporphyria[TI] liver[TI]


Komal Arora, M.D.
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Cite this page: Arora K. Erythropoietic protoporphyria. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/livererythropoieticpor.html. Accessed July 15th, 2019.
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

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Heme biosynthetic pathway

Gross images

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Explanted liver

Microscopic (histologic) description
  • Deep brown colored bile in canaliculi, bile ducts and Kupffer cells with red "Maltese cross" under polarized light
Microscopic (histologic) images

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Birefringence of
pigment deposits
due to protoporphyrin
crystals

Diffuse dark
discoloration
and micronodular
cirrhosis

Electron microscopy description
  • Starburst crystalline array
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