4A00.14 Hereditary angioedema

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2026-01

Hereditary angioedema is caused in the majority of cases by genetically determined low absolute (type I) or functional (type II) levels of C1 inhibitor, a plasma proteinase inhibitor involved in regulation of complement activation. It is characterised clinically by recurrent subcutaneous and/or submucosal oedema and can result in life-threatening laryngeal obstruction. Involvement of the digestive tract commonly causes abdominal pain. This and the absence of accompanying urticarial weals or itch distinguish it from the common form of angioedema, which is part of the spectrum of urticaria.

synonyms

• Hereditary angioedema
• Bannister disease, hereditary
• Familial angioedema
• HAE - [hereditary angioneurotic oedema]
• Hereditary angioneurotic oedema
• Quincke disease or oedema
• hereditary Quincke oedema
• Hereditary angioedema type I
• C1 esterase inhibitor [C1-INH] quantitative deficiency
• C1 esterase inhibitor deficiency - type 1
• HAE-I - [Hereditary angioedema type I]
• Hereditary angioneurotic oedema type 1
• deficiency of c1 esterase inhibitor
• Hereditary angioedema type II
• C1 esterase inhibitor [C1-INH] qualitative deficiency
• C1-INH - [C1 esterase inhibitor] deficiency
• HAE-II - [Hereditary angioedema type II]
• Hereditary angioneurotic oedema type 2
• Hereditary angioedema type III
• HAE-III - [Hereditary angioedema type III]
• Hereditary angioedema with FXII mutation
• Hereditary angioneurotic oedema type 3
• Inherited oestrogen-dependent angioedema
• Inherited oestrogen-dependent angioneurotic oedema
• Acute hereditary circumscribed oedema
• Acute hereditary essential oedema
• Hereditary giant urticaria
• Periodic hereditary oedema
• Hereditary allergic angioedema
• Hereditary angioneurotic oedema urticaria

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