Farber disease

Orpha code: 333OMIM code: 228000

Definition

A subcutaneous tissue disease 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.

Disease data
Classification

Disease

Synonyms
Choroba Farbera
Acid ceramidase deficiency
Niedobór ceramidazy
Farber lipogranulomatosis
ORPHA code
333
OMIM code
228000
ICD10 code
E75.2
ICD11 code
5C56.0Y

No additional description.

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