Hereditary angioedema

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C1 inhibitor deficiency → ↑bradykinin and C2b

Presentation

  • Non-inflammatory edema of the face, limbs, genitalia
  • Laryngeal edema
  • Abdominal pain (intestinal edema)
  • No urticaria
  • Follows infections, procedures or trauma

Diagnosis

  • C4
  • Normal C1q

Treatment

  • Acute: FFP, Ecallantide
  • Long-term: Androgens (danazol)
  • Icatibant

Differential diagnosis

  • Acquired angioedema (ACEi): C1q, presents later in life