Which clinical history findings are characteristic of acquired angioedema (AAE) due to C1 inhibitor (C1-INH) deficiency (C1-INH-AAE)?

Updated: May 20, 2020
  • Author: Amanda T Moon, MD; Chief Editor: William D James, MD  more...
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Apart from negative family history and age of onset, acquired angioedema cannot be distinguished clinically from hereditary angioedema.

Patients with acquired angioedema typically report no family history and onset is most commonly after the fourth decade.

Patients with acquired angioedema, similar to hereditary angioedema, report recurrent episodes of subcutaneous or submucosal edema at various locations, usually lasting 48-72 hours, but they can persist for up to 5 days in rare occasions. The edema is usually unresponsive to antihistamine therapy. The frequency of attacks is unpredictable and varies widely among affected individuals, as well as in the same individual. [10, 11]

Angioedema symptoms are referable to 3 prominent sites: subcutaneous tissues (eg, face, hands, arms, legs, genitals, buttocks); abdominal organs (eg, stomach, intestines, bladder), which may manifest as nausea, vomiting, and/or colicky pain and mimic a surgical emergency; and the upper airway (eg, larynx), which may result in laryngeal edema.

The most common reported symptom is nonpitting, nonpruritic cutaneous swelling of face and extremities.

Occasionally, acquired angioedema patients may experience heat and pain in the affected areas. Other symptoms of acquired angioedema may be related to underlying disorders, such as lymphoproliferative malignancies or connective tissue disease.

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