Orphanet: Omenn syndrome

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Omenn syndrome

Disease definition

Omenn syndrome (OS) is an inflammatory condition characterized by erythroderma, desquamation, alopecia, chronic diarrhea, failure to thrive, lymphadenopathy, and hepatosplenomegaly, associated with severe combined immunodeficiency (SCID; see this term).


Classification level: Disorder
  • Synonym(s):
    • Combined immunodeficiency with hypereosinophilia
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: D81.8
  • OMIM: 603554
  • UMLS: C2700553
  • MeSH: -
  • GARD: 8198
  • MedDRA: 10069097

Detailed information


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