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Angioedema (Without Urticaria)

(Figure 3.22A, B)
  • Deep form of urticaria with localized non-pitting edema
  • Presents with episodes of painful deep swelling of subcutaneous tissue (especially periorbital/lips), GI tract (abdominal pain) and upper respiratory tract (laryngeal edema), lasts > 24 h (usually few days)
  • Etiology: idiopathic, drug-related (NSAID, ACEI) or abnormality of C1 inhibitor (C1-INH)

    •    
     
    ACEI: results in unregulated generation of bradykinin
    		 
       

  • C1-INH: serine protease inhibitor, prevents spontaneous activation of complement system
  • Hereditary angioedema (HAE)
    • Autosomal dominant
    • C1-INH deficiency
      • Type 1: ↓ C1-INH level
      • Type 2: normal/↑ C1-INH level, but dysfunctional
    • Presents in 1st or 2nd decade
    • Labs: ↓ C4, ↓ C1-INH (level and/or function); C1q normal
    • Histology: perivascular lymphocytic infiltrate and dermal edema
    • Treatment: C1-INH concentrate during acute attack (of note, antihistamine, epinephrine, corticosteroid → not typically effective); fresh frozen plasma (FFP) before surgery; prophylactic treatment with attenuated androgens like danazol and stanazolol
  • Acquired C1 inhibitor deficiency (AAE)
    • Usually seen after fourth decade, no family history; due to destruction of C1-INH function through either immune complexes or autoantibodies
    • Presentation similar to HAE
      • Type 1: associated with lymphoproliferative disorders (i.e. B cell lymphoma, multiple myeloma, non-Hodgkin’s lymphoma) with significant amounts of immune complexes consuming C1q
      • Type II: associated with autoimmune phenomenon with autoantibodies to C1-INH molecule
    • Both AAE types with ↓ C1q (unlike HAE), ↓ C4/C2
    • Treatment: requires much higher amounts of C1-INH concentrate than in HAE during acute attack
   
 
TypeC1-INH LevelC1-INH FunctionC4C1q
 HAE, type 1 ↓ ↓ ↓ Normal
 HAE, type 2 Normal to ↑ ↓ ↓ Normal
 AAE, type 1 ↓ ↓ ↓ ↓
 AAE, type 2 ↓ ↓ ↓ ↓
 
   
Figure 3.22 A: Angioedema (Courtesy of Dr. Paul Getz) B: Hereditary angioedema (Reprint from Bork K, Barnstedt S. Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema. JADA 2003:1088–94. Copyright ©2003 American Dental Association. All rights reserved. Reprinted by permission)
Figure 3.22
A: Angioedema
(Courtesy of Dr. Paul Getz)
B: Hereditary angioedema
(Reprint from Bork K,
Barnstedt S. Laryngeal edema
and death from asphyxiation
after tooth extraction in four
patients with hereditary
angioedema.
JADA 2003:1088–94.
Copyright ©2003 American
Dental Association. All rights
reserved. Reprinted by
permission)





 

 

 

 

 

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