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Farber lipogranulomatosis
rareA rare sphingolipid disorder characterized by a spectrum of clinical signs ranging from the classical triad of painful and progressively deformed joints, subcutaneous nodules, and progressive hoarseness (due to laryngeal involvement) that presents in infancy, to varying phenotypes with respiratory and neurologic involvement.
- Identifiers
- MONDO:0009218 OMIM:228000Orphanet:333
- Part of
- ASAH1-related sphingolipidosis
- Also known as
- acid ceramidase deficiency · Farber disease · Farber's disease · FRBRL · N-LAURYLSPHINGOSINE deacylase deficiency
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