Farber’s disease, Farber’s lipogranulomatosis

Farber’s disease, Farber’s lipogranulomatosis /fär′bərz/ [Sidney Farber, American pediatrician, 1903–1973] , a lysosomal storage disease of ceramide metabolism resulting from defective ceramidase. The disease is marked by hoarseness; aphonia; a brownish desquamating dermatitis that begins at about 3 months of age; foam cell infiltration of bones and joints that causes deformations; granulomatous reaction in lymph nodes, heart, lungs, and kidneys; and psychomotor delay. Also called ceramidase deficiency.