Hereditary angioedema is caused by an absolute deficiency or the functional inactivity of C1 esterase inhibitor in plasma. A precise diagnosis is important because, unlike allergic forms of mucocutaneous edema, this condition does not respond to epinephrine, antihistamines, or corticosteroids. We report the case of a 24-year-old man who experienced an acute attack after he had stopped taking his prophylactic medication.
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