4A00.15

ICD-11 MMS 2026-01

Acquired angioedema

Description

Acquired angioedema is clinically similar to hereditary angioedema and is not associated with urticaria. It may be associated with a lymphoproliferative disorder (type I) or may be an isolated phenomenon due to an autoantibody directed against C1 inhibitor (type II).

Index terms

  • Acquired angioedema
  • AAE - [Acquired angioneurotic oedema]
  • Acquired angioneurotic oedema
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  • Acquired angioedema type I
  • AAE - [Acquired angioedema type I]
  • Acquired angioedema type II
  • AAE-II - [Acquired angioedema type II]

Exclusions from above levels

  • 3A10.Y – Atypical haemolytic uraemic syndrome
  • 3A21.0 – Paroxysmal nocturnal haemoglobinuria
  • 02 – Neoplasms
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A00.10 – Immunodeficiency with an early component of complement deficiency
  • 4A00.11 – Immunodeficiency with a late component of complement deficiency
  • 4A00.12 – Immunodeficiency with factor B deficiency
  • 4A00.13 – Immunodeficiency with factor D anomaly
  • 4A00.14 – Hereditary angioedema
  • 4A00.1Y – Other specified defects in the complement system
  • 4A00.1Z – Defects in the complement system, unspecified

Foundation entity

Acquired angioedema – http://id.who.int/icd/entity/1078767412

Part of: 4A00.1: Defects in the complement system › 4A00: Primary immunodeficiencies due to disorders of innate immunity › 04: Diseases of the immune system