Table 1 Clinical and diagnostic features of various types of angioedema

Histamine-mediated

Allergic angioedema

Angioedema usually accompanied by urticaria and sometimes anaphylaxis; may be pruritic; associated with exposure to allergens; attacks last for 24–48 h; responsive to antihistamines or corticosteroids

Angioedema with urticarial vasculitis

Angioedema accompanied by urticaria; there may be petechiae or purpura after swelling resolves; symptoms of underlying vasculitis

Bradykinin-mediated

Hereditary angioedema types I and II

Recurrent attacks without urticaria; erythema marginatum is a cardinal finding; onset in childhood or young adulthood, worsening at puberty; family history in 75% of patients; attacks unresponsive to antihistamines or corticosteroids

Hereditary angioedema type III

Associated with mutations in factor XII; more common in women; may be estrogen dependent; typical onset after childhood; face, tongue, extremity involvement is more frequent than abdominal; recurrent tongue swelling is cardinal symptom; more disease-free intervals than in HAE types I and II; family history of angioedema; attacks unresponsive to antihistamines or corticosteroids

Acquired angioedema

Attacks similar to HAE; onset in middle age or later; no family history; attacks unresponsive to antihistamines or corticosteroids

ACE inhibitor-induced angioedema

History of ACE inhibitor use; no urticaria; face and tongue most frequent sites; more common in blacks and smokers; patients usually can tolerate ARBs

Not mediated by histamine or bradykinin

Idiopathic angioedema

Angioedema sometimes accompanied by urticaria; swelling may persist for up to 48 h; attacks may occur daily; responsive to antihistamines or corticosteroids

Pseudoallergic angioedema

Urticaria typically present; usually class-specific reaction; thought to be mediated by cysteinyl-leukotrienes; includes NSAID-induced angioedema, which occurs because of cyclooxygenase inhibition and subsequent release of cysteinyl-leukotrienes