Wolmans xanthomatosis

From Biology-Online Dictionary
Jump to: navigation, search

Wolman's xanthomatosis --> cholesterol ester storage disease

A rare benign adult form of inherited lysosomal lipid storage disease that is due to deficiency of acid lipase. It results in an accumulation of neutral lipids, particularly cholesterol esters, within cells (particularly leukocytes, fibroblasts, and liver cells). It is an allelic variant of wolman disease.