title: Hereditary angioedema: a case of near fatal laryngeal swelling in a 41-year-old woman.
abstract: Hereditary angioedema (HAE) is characterized by nonpitting, nonpruritic subcutaneous, or submucosal edema that may be accompanied by a nonpruritic serpentine erythematous rash. The swelling in this autosomal dominantly inherited disorder is mediated by uncontrolled localized bradykinin  production. The extremities, face, gastrointestinal tract, genitalia, larynx, and trunk are commonly involved. Nearly one-third of patients who are not treated may suffer asphyxiation from a laryngeal attack. HAE symptoms overlap with other conditions that cause swelling. An accurate diagnosis  is therefore crucial when considering therapy. Recently approved treatments for HAE swelling attacks include C1 esterase inhibitor concentrate and ecallantide. This case describes a 41-year-old woman who was treated ineffectively for more than 20 years, was switched to an effective therapy,  but suffered near fatal laryngeal edema due to a missed dose of the effective therapy. The case underscores the need for increased awareness of HAE among clinicians and the importance of good compliance to therapy among patients with HAE.
authors.affiliation: Department of Internal Medicine/Allergy Division, University of Texas Southwestern Medical School-Dallas, Dallas, Texas, USA.
authors.forename: William R
authors.initials: WR
authors.lastname: Lumry
mesh_terms: D000328:Adult; D054179:Angioedemas, Hereditary / Q000150:complications* / Q000188:drug therapy*; D003174:Complement C1 Inactivator Proteins / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007362:Intensive Care Units / Q000191:economics; D007442:Intubation, Intratracheal; D007819:Laryngeal Edema / Q000209:etiology* / Q000628:therapy; D055118:Medication Adherence; D016896:Treatment Outcome
chemical_list: D003174:Complement C1 Inactivator Proteins