The severe infantile form of inherited lysosomal lipid storage diseases due to deficiency of acid lipase (). It is characterized by the accumulation of neutral lipids, particularly in leukocytes, fibroblasts, and hepatocytes. It is also known as Wolman's xanthomatosis and is an allelic variant of .
Xanthomatose familiale primitive /
Déficit en lipase acide lysosomiale /
Déficit en lipase acide lysosomale type Wolman /