Idiopathic peliosis hepatis

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Idiopathic peliosis hepatis

ORPHA:480524Disease

Also called Idiopathic peliosis hepatitis

What it is

A rare vascular liver disease characterized by widespread or focal cystic dilatation of sinusoidal blood-filled spaces of the liver without any known cause. Lesions can vary in diameter between few millimeters and several centimeters. The condition may remain asymptomatic or manifest with complications including rupture and intraperitoneal hemorrhage, hepatomegaly, portal hypertension, cholestasis, and liver failure.

Key facts

Age of onset
Adult, Childhood
Inheritance
Not applicable
Classified as
Disease

ICD-10 codes

K76.4filed under a broader ICD-10 category

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0018809UMLS C5680101

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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