II. Pathophysiology

  1. See Urticaria

III. Signs

  1. May be associated with Anaphylaxis
  2. May occur with Urticaria
  3. Nonpitting subcutaneous swelling
    1. Well demarcated
    2. Distribution
      1. Face
      2. Hands
      3. Buttocks
      4. Genitalia
      5. Abdomen
      6. Laryngeal Angioedema (treat as Anaphylaxis)
  4. Associated findings
    1. Wheezing or Stridor
    2. Fever
    3. Lymphadenopathy

IV. Causes: Specific Types

  1. Aspirin or NSAID reaction
  2. Penicillin Allergy
  3. Anaphylactoid Reaction to Radiocontrast
    1. Immediate reaction to small dose (does not require presensitization)
    2. Contrast with Anaphylaxis which is IgE mediated and requires allergen presensitization
  4. ACE Inhibitor
    1. ACE-Inhibitor induced Angioedema is not an Allergic Reaction (unlike typical Angioedema)
      1. Related to bradykinin accumulation
      2. Does not respond to typical Angioedema management (e.g. Corticosteroids, Antihistamines)
    2. More common in african american patients by factor of 2-4 fold
    3. Reaction can occur months to years after starting an ACE Inhibitor
    4. Treatment is withdrawal of medication and supportive care
      1. ACE-Inhibitor Angioedema will recur with ARB agents in 8% of patients
      2. Reactions may be severe and life threatening with complete airway closure

V. Evaluation

  1. See Urticaria

VI. Management

  1. Chronic Angioedema
    1. See Urticaria
  2. Acute Angioedema (especially laryngeal Angioedema)
    1. See Allergic Reaction and Anaphylaxis
    2. Severe, airway compromising hereditary Angioedema (and off label for ACE Inhibitor Angioedema)
      1. C1 Human Esterase Inhibitor (e.g. Berinert)
        1. Dose: 20 IU/kg IV for 1 dose ($3000 per dose)
      2. Bradykinin B2 inhibitor (Icatibant or ecallantide)
        1. Effective in one RCT for ACE Inhibitor Angioedema (symptoms resolved 19 hours faster)
          1. Dose: 30 mg SQ (and may repeat in 6 hours if inadequate effect, $10,000/dose)
          2. Bas (2015) N Engl J Med 372(5):418-25 +PMID:25629740 [PubMed]
        2. Icatibant did not show benefit in ACE Inhibitor Angioedema subsequent Phase III trial
          1. Swaminathan and Wilkerson in Herbert (2016) EM:Rap 16(3): 3-4
      3. Fresh Frozen Plasma (FFP)
        1. Indicated if the above agents (C1 and B2) are unavailable in a severe reaction
        2. Theoretical risk of exacerbating Angioedema (due to complement in FFP)
        3. Hassen (2013) J Emerg Med 44(4):764-72 +PMID:23114109 [PubMed]

VII. References

  1. Frank in Goldman (2000) Cecil Medicine, p. 1440-5
  2. Kaplan in Middleton (1998) Allergy, p. 1104-18
  3. Habif (1996) Clinical Dermatology, p. 122-47
  4. Cicardi (2014) Intern Emerg Med 9(1):85-92 +PMID: 24002787 [PubMed]
  5. Greaves (2000) J Allergy Clin Immunol 105:664-72 [PubMed]
  6. Muller (2004) Am Fam Physician 69(5):1123-8 [PubMed]

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Ontology: Angioedema (C0002994)

Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (CHV) Recurring attacks of transient edema suddenly appearing in areas of the skin or mucous membranes
Definition (NCI) Rapid swelling of the deep layers of the skin due to transient vascular leakage of serous fluid.(NICHD)
Definition (MSH) Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA. Angioedema often occurs in the face, lips, tongue, and larynx.
Concepts Pathologic Function (T046)
MSH D000799
ICD10 T78.3
SnomedCT 269433002, 157756002, 400075008, 41291007, 82966003
English Angioedemas, Angioneurotic Edemas, Edemas, Angioneurotic, Giant Urticarias, Quincke Edema, Quincke's Edema, Quinckes Edema, Urticaria, Giant, Urticarias, Giant, Edema, Quincke's, ANGIOEDEMA, ANGIONEUROTIC EDEMA, ANGIONEUROTIC OEDEMA, Edema, Angioneurotic, GIANT HIVES, GIANT URTICARIA, Giant Urticaria, QUINCKE'S EDEMA, QUINCKE'S OEDEMA, Urticaria giant, Hives giant, Oedema angioneurotic, Giant hives, Edema angioneurotic, Edema Quincke's, Angio-edema, Oedema Quincke's, Angio-edema-urticaria, Urticaria-angio-edema, Angioneurotic Edema, Angioedema, angioedema (diagnosis), angioedema, Angioedema and urticaria, Angioedema [Disease/Finding], angioedema urticaria, Oedema;angioneurotic, quincke edema, quincke's edema, urticaria angioedema, angio edema, angio oedema urticaria, angio oedema, giant hives, quincke's disease, Giant urticaria, Angioneurotic edema, Quincke's edema, Angioneurotic oedema, Quincke's oedema, Quincke's disease, Angio-oedema, Angio-oedema-urticaria, Urticaria-angio-oedema, Giant urticaria (disorder), Angioedema (disorder), Angioedema-urticaria, Urticaria-angioedema, giant urticaria, circumscribed; edema, Quincke; edema, Quincke, edema; Quincke, edema; angioneurotic, edema; circumscribed, giant; urticaria, angioneurotic; edema, urticaria; giant, Angioedema, NOS, Angioneurotic edema, NOS, angioneurotic edema, angioneurotic oedema, Edema;angioneurotic
French URTICAIRE GEANTE, Angio-oedème, Urticaires géantes, Oedème de Qincke, Oedème angionévrotique, Angioedème, Oedème de Quincke, ERUPTION ALLERGIQUE GEANTE, OEDEME ANGIONEUROTIQUE, OEDEME ANGIONEVROTIQUE, OEDEME DE QUINCKE, Angioedèmes, Angioedèmes et urticaires, Urticaire géante, Oedème angioneurotique
Portuguese EDEMA ANGIONEUROTICO, ANGIOEDEMA, EDEMA DE QUINCKE, URTICARIA GIGANTE, Angiedema, Edema angioneurótico, Urticária gigante, Edema angioneurõtico, Angioedema, Angiodemas, Angioedema e urticária, Edema de Quincke, Urticária Gigante
Spanish EDEMA ANGIONEUROTICO, ANGIOEDEMA, EDEMA DE QUINCKE, URTICARIA GIGANTE, Edema angioneurótico, Urticaria gigante, Angioedema, angioedema (trastorno), angioedema, edema angioneurótico, edema de Quincke, urticaria gigante (trastorno), urticaria gigante, Angioedema y urticaria, Angioedemas, Edema de Quincke, Urticaria Gigante
Italian Angioedema ed orticaria, Angioedemi, Edema angioneurotico, Edema di Quincke, Orticaria gigante, Angioedema
Japanese 血管浮腫および蕁麻疹, ケッカンフシュオヨビジンマシン, ケッカンシンケイセイフシュ, クインケフシュ, キョダイジンマシン, ケッカンフシュ, Quincke浮腫, 血管神経性浮腫, クインケ浮腫, 巨大じんま疹, 巨大浮腫, 巨大蕁麻疹, 皮膚浮腫, 脈管神経性水腫, 脈管神経性浮腫, 脈管運動神経性水腫, 脈管運動神経性浮腫, 血管浮腫, 血管神経性水腫, 血管運動神経性水腫, 血管運動神経性浮腫, 遊走性水腫, じんましん-巨大, 巨大じんましん, 蕁麻疹-巨大, 血管性浮腫
Dutch netelroos (galbulten), oedeem, Quincke, oedeem angioneurotisch, angio-oedeem, angioneurotisch oedeem, giga urticaria, reuzenurticaria, Quincke; oedeem, angioneurotisch; oedeem, circumscript; oedeem, oedeem; Quincke, oedeem; angioneurotisch, oedeem; circumscript, reuzen; urticaria, urticaria; reuzen, angio-oedeem en urticaria, angio-oedemen, Angio-oedeem, Angioneurotisch oedeem, Oedeem, angioneurotisch, Quincke-oedeem, Urticaria, reuzen-
German Quincke-Oedem, Riesenurtikaria, angioneurotisches Oedem, Oedem angioneurotisch, Oedem Quinke, Quaddeln riesig, Angiooedem, Riesenquaddeln, ANGIOOEDEM, Angioneurotisches Oedem, OEDEM ANGIONEUROTISCH, QUINCKE OEDEM, URTICARIA GIGANTEA, URTIKARIA GIGANTEA, Angiooedem und Urtikaria, Angiooedeme, Urticaria gigantea, Angioneurotisches Ödem, Angioödem, Quincke-Ödem
Finnish Angioedeema
Russian KRAPIVNITSA GIGANTSKAIA, ANGIONEVROTICHESKII OTEK, АНГИОЭДЕМА, ANGIOEDEMA, АНГИОНЕВРОТИЧЕСКИЙ ОТЕК, KVINKE OTEK, КВИНКЕ ОТЕК, КРАПИВНИЦА ГИГАНТСКАЯ
Swedish Angioödem
Czech Angioedémy, Kopřivka tvořená rozsáhlými kopřivkovými pupeny, Rozsáhlá kopřivka, Velkopupenová kopřivka, Quinckeho edém, Angioneurotický edém, Angioedém, Angioedém a kopřivka, Kopřivka tvořená rozsáhlými pomfy, angioedém, angioneurotický edém
Korean 혈관신경성 부종
Polish Obrzęk naczynioruchowy, Obrzęk naczyniowo-nerwowy, Obrzęk Quinckego
Hungarian Angio-oedemák, Quincke oedema, Angio-oedema és urticaria, Angio-oedema, Quincke-vizenyő, Óriás urticaria, Quincke-oedema, Angioneurotikus oedema, Oedema, angioneurotica, Óriás csalánkiütés, Urticaria óriás, angioneurotikus oedema, Angiooedema, ér eredetű vizenyő, Oedema angioneurotica, Csalánkiütés, óriás, Quincke-edema
Norwegian Quinckes ødem, Angionevrotisk ødem, Angioødem, Urticaria gigantea