Papillomatosis of the Gallbladder in Metachromatic Leukodystrophy

A 7-year-old boy with typical metachromatic leukodystrophy was found to have a marked diffuse hyperplasia of the mucosa of the gallbladder. The stroma of the villi revealed a large number of macrophages with metachromatic material which also contained cholesterol. An increased biliary excretion of sulfatides might have led to deposition and an enlargement of the mucosal surface. An extensive papillomatosis associated with metachromatic leukodystrophy has been described only once and is not known in other diseases with an inborn error of metabolism.