Orphanet: Acquired angioedema

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Acquired angioedema

Disease definition

A rare disease characterized by the occurrence of transitory and recurrent subcutaneous and/or submucosal edemas resulting in swelling and/or abdominal pain due to an acquired C1 inhibitor (C1-INH) deficiency.


Classification level: Group of disorders
  • Synonym(s):
    • AAE
    • Acquired C1 inhibitor deficiency
    • Acquired angioneurotic edema
    • Acquired bradykinine-induced angioedema
    • Acquired non histamine-induced angioedema
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: T78.3
  • OMIM: 300909
  • UMLS: C2931758
  • MeSH: C538173
  • GARD: 8605
  • MedDRA: -
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