Farber Lipogranulomatosis

**Definition:** A sphingolipidosis subtype that is characterized by the histological appearance of granulomatous deposits in tissues. It results from the accumulation of CERAMIDES in various tissues due to an inherited deficiency of ACID CERAMIDASE.

**Tree numbers:** - C10.228.140.163.100.435.825.250 - C16.320.565.189.435.825.250 - C16.320.565.398.641.803.325 - C16.320.565.595.554.825.250 - C18.452.132.100.435.825.250 - C18.452.584.563.641.803.325 - C18.452.648.189.435.825.250 - C18.452.648.398.641.803.325 - C18.452.648.595.554.825.250

**Synonyms:** - Acid Ceramidase Deficiency - Farber Disease - Farber's Disease - N-Laurylsphingosine Deacylase Deficiency - Ceramidase Deficiency