Hereditary angioedema is a relatively rare genetic disease with an autosomal dominant inheritance pattern. It is caused by a quantitative or qualitative deficiency of Complement 1 inhibitor (C1-INH). Patients develop episodes of angioedema under the skin, cramping abdominal pain, or laryngeal edema, which can be fatal. The disease does not typically respond to antihistamine, corticosteroid, or epinephrine treatments. The following two cases in this chapter will illustrate the presentation, diagnosis, and management of the disease.
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