Farber disease

dis·sem·i·nat·ed lip·o·gran·u·lo·ma·to·sis

[MIM*228000]

a form of mucolipidosis that develops soon after birth because of a deficiency of ceramidase; characterized by swollen joints, subcutaneous nodules, lymphadenopathy, and accumulation in lysosomes of affected cells of PAS-positive lipid consisting of ceramide.

Synonym(s): Farber disease, Farber syndrome

Farber disease

(far'ber)

[Sidney Farber, U.S. pediatric pathologist, 1903–1973]

A rare autosomal recessive lysosomal storage disease in which abnormal by-products of lipid metabolism accumulate in the brain. It is one of the lipid storage diseases, which include Gaucher's disease and Niemann-Pick disease.

Synonym: ceramidase deficiency

Farber,

Sidney, U.S. pediatric pathologist, 1903-1973.

Farber disease - a form of mucolipidosis, developing soon after birth because of deficiency of ceramidase. Synonym(s): disseminated lipogranulomatosis; Farber syndrome

Farber syndrome - Synonym(s): Farber disease