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Genetic and Rare Diseases Information Center (GARD)

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Acquired angioedema


Other Names for this Disease

  • Acquired C1 inhibitor deficiency
  • Angioedema, acquired
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Overview

Acquired angioedema (AAE) is a rare condition characterized by recurrent episodes of swelling (edema). Affected people may have swelling of the face, lips, tongue, limbs, and genitals. Sometimes there is abdominal pain due to edema of the gastrointestinal mucosa, and life-threatening edema of the upper respiratory tract.[1] Swelling episodes may be triggered by mild trauma (e.g., dental work), viral illness, cold exposure, pregnancy, ingestion of certain foods, or emotional stress.[2] There are two forms of AAE: type I and type II. AAE type I is most commonly associated with B-cell lymphoproliferative disorders and consumption of C1-INH.  AAE type II is an autoimmune condition associated with the presence of an autoantibody against C1-INH.[1][3] Treatment aims to control episodes and normalize C1-INH levels.[3] When possible, the underlying disorder should be treated.[1]
Last updated: 8/25/2014

References

  1. Ru'aa Al Harithy. Acquired Angioedema. Medscape Reference. July 31, 2014; http://emedicine.medscape.com/article/1048887-overview. Accessed 8/25/2014.
  2. Peter J. Delves. Hereditary and Acquired Angioedema. Merck Manuals. March, 2014; http://www.merckmanuals.com/professional/immunology_allergic_disorders/allergic_autoimmune_and_other_hypersensitivity_disorders/hereditary_and_acquired_angioedema.html. Accessed 8/25/2014.
  3. Laurence Bouillet. Acquired Angioedema. Orphanet. August, 2011; Accessed 8/25/2014.
Your Questions Answered
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Basic Information

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Merck Manual for health care professionals provides information on Acquired angioedema.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Acquired angioedema. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Acquired C1 inhibitor deficiency
  • Angioedema, acquired
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.