Hereditary angioedema attack – case presentation

Abstract

Hereditary angioedema, or inherited C1-inhibitor esterase deficiency, is a rare genetic disorder, characterized by recurrent episodes of isolated angioedema, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. Although swelling resolves spontaneously in two to five days in the absence of treatment, angioedema is often temporarily debilitating, and laryngeal angioedema may cause fatal asphyxiation. Dental surgery and intubation are the major triggers for hereditary angioedema attacks. Typically, laryngeal edema develops 24 to 48 hours after the onset of prodromal signs. However, rapidly evolving, fulminant laryngeal edema cases have been described. We present the case of a 42-year-old male patient, with a medical history of hereditary angioedema, included in the National Program of Hereditary Angioedema, who was referred to the Allergy Department of the „Prof. Dr. Octavian Fodor“ Institute of Gastroenterology and Hepatology, Cluj-Napoca, for dysphagia, dysphonia, facial and right laterocervical edema, events that started about one hour after a dental surgery for which he received premedication with a C1-inhibitor esterase recombinant. Physical examination revealed facial and right laterocervical angioedema, edema of the oral and pharyngeal mucosa, dysphonia, tachycardia, and decreased SaO2. Subcutaneous administration of icatibant significantly improved the signs and symptoms. Laryngeal edema is a life-threatening complication of hereditary angioedema attacks. Dental surgery and tracheal intubation are major risk factors for attacks. Although premedication with C1-inhibitor esterase recombinant appears to have prevented such events, this was not the case for our patient. Also, hereditary angioedema signs and symptoms typically consolidate in one up to two days after the prodromal phase. In the present case, the clinical picture had a rapid evolution. The treatment with bradikinin B2 receptor antagonists brought rapid efficient clinical benefit.