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Angioedema

Angioedema is a topic covered in the 5-Minute Emergency Consult.

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Basics

Description

  • Nonpruritic, well-demarcated, nonpitting edema of the dermis
  • Due to the release of inflammatory mediators that cause dilation and increased permeability of capillaries and venules:
    • Mast cell mediated
    • Kinin related from the generation of bradykinin and complement-derived mediators
  • Similar in pathologic basis to urticaria except that affected tissue lies deeper:
    • Urticaria affects superficial tissue and causes irritation to mast cells and nerves in the epidermis leading to intense itching.
    • Angioedema occurs in deeper layers, which have fewer mast cells and nerves, therefore causing less itching.
    • Angioedema can affect tissues other than the skin, for example, the gut.
    • Angioedema may be seen in a mixed picture along with urticaria
  • Hereditary and acquired etiologies are due to deficiencies in the quantity and/or function of C1-INH rather than classic hypersensitivity reactions
  • Hereditary angioedema (HAE):
    • An autosomal dominant disorder caused by a deficiency of C1-INH
    • The prevalence of HAE is estimated to range from 1:10,000 to 1:150,000.
    • C1-INH has a regulatory role in the contact, fibrinolytic, and coagulation pathways.
    • Deficiency results in unregulated activity of the vasoactive mediators bradykinin, kallikrein, and plasmin.
    • More than 100 mutations of the C1-INH gene have been reported.
    • Type 1: Decreased expression of C1-INH
    • Type 2: Normal plasma levels of C1-INH but the protein is dysfunctional
    • Type 3: Mutation in coagulation factor XII resulting in increased kinin production:
      • Symptoms increased by estrogen-containing medications
    • Episodes occur in all 3 types when inflammation, trauma, or other factors lead to depletion of C1-INH.
  • Acquired angioedema:
    • Normal quantities and function of C1-INH
    • Type 1 is associated with lymphoproliferative diseases and caused by consumption of the C1-INH protein by malignant cells.
    • Type 2 is autoimmune caused by circulating antibodies that inactivate C1-INH.
  • ACE inhibitor–induced angioedema:
    • Accounts for 2030% of all angioedema cases presenting to ER reported in 0.1–2.2% of patients taking ACE inhibitors
    • Usually occurs during the 1st mo of taking the medication, however the 1st event may occur spontaneously after many years.

Etiology

  • Kinin-related etiologies:
    • HAE
    • Acquired angioedema:
      • Lymphoproliferative
      • Autoimmune
    • ACE inhibitor induced
  • Mast cell–mediated etiologies:
    • Food allergies:
      • Additives
      • Nuts
      • Eggs/milk
      • Shellfish
      • Soy/wheat
    • Drug allergies:
      • Aspirin
      • NSAID
      • Antihypertensives
      • Narcotics
      • Oral contraceptives
    • Insect stings
    • Physically induced:
      • Cold/heat
      • Exercise/trauma/vibrations
      • Stress
      • UV light
  • Hypereosinophilic syndromes such as Gleich syndrome
  • Thyroid autoimmune disease
  • Idiopathic recurrent AE

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Citation

Rosen, Peter, et al., editors. "Angioedema." 5-Minute Emergency Consult, 5th ed., Lippincott Williams & Wilkins, 2016. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307295/all/Angioedema.
Angioedema. In: Rosen P, Shayne P, Barkin AZ, et al, eds. 5-Minute Emergency Consult. 5th ed. Lippincott Williams & Wilkins; 2016. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307295/all/Angioedema. Accessed April 24, 2019.
Angioedema. (2016). In Rosen, P., Shayne, P., Barkin, A. Z., Wolfe, R. E., Hayden, S. R., Barkin, R. M., & Schaider, J. J. (Eds.), 5-Minute Emergency Consult. Available from https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307295/all/Angioedema
Angioedema [Internet]. In: Rosen P, Shayne P, Barkin AZ, Wolfe RE, Hayden SR, Barkin RM, Schaider JJ, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2016. [cited 2019 April 24]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307295/all/Angioedema.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Angioedema ID - 307295 ED - Rosen,Peter, ED - Shayne,Philip, ED - Barkin,Adam Z, ED - Wolfe,Richard E, ED - Hayden,Stephen R, ED - Barkin,Roger M, ED - Schaider,Jeffrey J, BT - 5-Minute Emergency Consult UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307295/all/Angioedema PB - Lippincott Williams & Wilkins ET - 5 DB - Emergency Central DP - Unbound Medicine ER -