Disseminated lipogranulomatosis

[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. SYN: Farber disease, Farber syndrome.

Chemwatch
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