The patient had a full recovery and was discharged home on androgen maintenance therapy. Discussion Early recognition of hereditary and acquired angioedema in the ED is important because significant iatrogenic morbidity may result if the diagnosis is not established. The diagnosis of these disorders is aided by a detailed past medical and family history. Hereditary and acquired angioedema are clinically characterized by recurrent episodes of angioedema involving any part of the body. Angioedema involving the GI tract may mimic an acute abdomen, resulting in unnecessary surgery and increased morbidity.
Hereditary angioedema is inherited as an autosomal dominant condition for which there are two forms.
One form is manifest by the absence of C1 esterase inhibitor, and the other form is manifest by normal levels of C1 esterase inhibitor, but the enzyme is dysfunctional. The absence of, or dysfunction of, C1 esterase inhibitor allows uncontrolled activation of the complement cascade, resulting in angioedema. The diagnosis is usually established by a family history of angioedema in a symptomatic patient, and the diagnosis is confirmed by biochemical C 1 esterase inhibitor deficiency or functional deficiency. Acquired forms of angioedema are associated with an underlying hematologic malignancy or immune complex disease, resulting in activation of C1Q in the complement cascade. The acquired and hereditary forms can be differentiated from each other by quantification of C1Q levels.