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Table 3 Comparison of HAE and AAE [23, 33]

From: Urticaria and angioedema

 

Family history

Complement levels/laboratory findings

C4

C1-INH antigen

C1-INH function

HAE-1

HAE-2

Yesa

↓

↓

↓

Normal or ↑

↓

↓

HAE-nC1-INH

    

 FXII mutation

Yes

Normal

Normal

Normal

 Unknown cause

    

AAE

No

↓

Normal or ↓

↓

  1. Adapted from Betschel et al. [23], Cicardi et al. [33]
  2. aIn approximately 25% of patients, no family history is identified; the disorder results from spontaneous mutation of the C1 inhibitor gene