Acquired angioedema

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Acquired angioedema

ORPHA:91385Clinical group

Also called AAE · Acquired C1 inhibitor deficiency · Acquired angioneurotic edema · Acquired bradykinine-induced angioedema · Acquired non histamine-induced angioedema

What it is

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.

Key facts

Prevalence
1-9 / 1 000 000 (Denmark)
Age of onset
Adult
Inheritance
Not applicable
Classified as
Clinical group

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes reported in subtypes

XPNPEP2

Orphanet records this gene on 1 more specific entry under this disorder, not on this entry itself:

A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.

Cross-references

GARD 8605MEDDRA 10081035MESH C538173MONDO 0019624OMIM 300909UMLS C2931758

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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