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Descriptor English: Farber Lipogranulomatosis
Descriptor Spanish: Lipogranulomatosis de Farber
Descriptor lipogranulomatosis de Farber
Scope note: Subtipo de esfingolipidosis que se caracteriza por la apariencia histológica de sus depósitos granulomatosos en los tejidos. Es el resultado de la acumulación de CERAMIDAS en diversos tejidos debido a una deficiencia heredada de CERAMIDASA ÁCIDA. 
Descriptor Portuguese: Lipogranulomatose de Farber
Descriptor French: Lipogranulomatose de Farber
Entry term(s): Acid Ceramidase Deficiencies
Acid Ceramidase Deficiency
Ceramidase Deficiencies
Ceramidase Deficiency
Ceramidase Deficiency, Acid
Deficiencies, Ceramidase
Deficiencies, N-Laurylsphingosine Deacylase
Deficiency, Acid Ceramidase
Deficiency, Ceramidase
Deficiency, N-Laurylsphingosine Deacylase
Disease, Farber's
Diseases, Farber's
Farber Disease
Farber's Disease
Farber's Diseases
Farbers Disease
Lipogranulomatosis, Farber
N Laurylsphingosine Deacylase Deficiency
N-Laurylsphingosine Deacylase Deficiencies
N-Laurylsphingosine Deacylase Deficiency
Tree number(s): 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
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D055577
Scope note: 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.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Lipid Metabolism (1952-1976)
Lipid Metabolism, Inborn Errors (1987-2008)
Lipidoses (1976-2008)
Sphingolipidoses (1992-2008)
Public MeSH Note: 2009
History Note: 2009
DeCS ID: 53092
Unique ID: D055577
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2009/01/01
Date of Entry: 2008/07/08
Revision Date: 2013/07/08
Farber Lipogranulomatosis - Preferred
Concept UI M0518626
Scope note 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.
Preferred term Farber Lipogranulomatosis
Entry term(s) Acid Ceramidase Deficiencies
Acid Ceramidase Deficiency
Ceramidase Deficiencies
Ceramidase Deficiency
Ceramidase Deficiency, Acid
Deficiencies, Ceramidase
Deficiencies, N-Laurylsphingosine Deacylase
Deficiency, Acid Ceramidase
Deficiency, Ceramidase
Deficiency, N-Laurylsphingosine Deacylase
Disease, Farber's
Diseases, Farber's
Farber Disease
Farber's Disease
Farber's Diseases
Farbers Disease
Lipogranulomatosis, Farber
N Laurylsphingosine Deacylase Deficiency
N-Laurylsphingosine Deacylase Deficiencies
N-Laurylsphingosine Deacylase Deficiency



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