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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