Acquired Angioedema Due to C1 Inhibitor Deficiency Clinical Presentation

Updated: May 20, 2020
  • Author: Amanda T Moon, MD; Chief Editor: William D James, MD  more...
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Presentation

History

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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Physical Examination

The clinical presentation is not distinguishable from hereditary angioedema. Physical signs include overt, noninflammatory swelling of the skin and mucous membranes. Although urticaria does not usually occur, occasionally, erythema or mild urticarial eruptions may precede the edema.

When looking at reports of different rates of recurrence at specific sites, hereditary angioedema has a tendency to present with more extremity involvement than does acquired angioedema, which presents more with face involvement than limb involvement. Acute abdominal pain due to edema of the gastrointestinal mucosa is less reported in acquired angioedema (30-50%) when compared with hereditary angioedema, in which it is reported in up to 80%. [10, 11]

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