Xanthomatous neuropathy of liver

A 36-year-old man with juvenile-onset diabetes mellitus developed cholestatic hepatitis of unknown cause, possibly drug-induced. He remained jaundiced for four months, until he died unexpectedly. At autopsy, severe and macroscopically identifiable xanthomatous neuropathy was found in the liver; it involved the unmyelinated nerves in the hilus and in all portal tracts examined. Microscopic evidence of minimal extrahepatic involvement of unmyelinated nerves was also found. Somatic nerves were affected by diabetic neuropathy but not by xanthomatous neuropathy. The condition seems to be a rare complication of diseases that are characterized by accumulations of lipids.