Acquired angioedema

J Am Acad Dermatol. 1997 Apr;36(4):611-5. doi: 10.1016/s0190-9622(97)70250-0.


Acquired angioedema (AAE) is a rare disorder that has been categorized into two forms, AAE-I and AAE-II. AAE-I is associated with other diseases, most commonly B-cell lymphoproliferative disorders. AAE-II is defined by the presence of an autoantibody directed against the C1-inhibitor molecule. Differentiating AAE-I from AAE-II is vital because different therapeutic interventions are required for each type. This review summarizes the clinical aspects, pathophysiology, and management of AAE compared with the types of hereditary angioedema.

Publication types

  • Review

MeSH terms

  • Angioedema* / complications
  • Angioedema* / pathology
  • Angioedema* / physiopathology
  • Angioedema* / therapy
  • Complement C1 Inactivator Proteins / genetics
  • Humans


  • Complement C1 Inactivator Proteins