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Hereditary angioedema: A case of near fatal laryngeal swelling in a 41-year-old woman

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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.

Keywords: Angioedema; C1 esterase inhibitor; C1 inhibitor; bradykinin; ecallantide; edema; hereditary; inherited; laryngeal edema; swelling

Document Type: Research Article

DOI: http://dx.doi.org/10.2500/aap.2011.32.3465

Affiliations: Department of Internal Medicine/Allergy Division, University of Texas Southwestern Medical School—Dallas, Dallas, Texas, USA

Publication date: September 1, 2011

More about this publication?
  • Allergy and Asthma Proceedings is a peer reviewed publication dedicated to distributing timely scientific research regarding advancements in the knowledge and practice of allergy, asthma and immunology. Its primary readership consists of allergists and pulmonologists.

    The goal of the Proceedings is to publish articles with a predominantly clinical focus which directly impact quality of care for patients with allergic disease and asthma.

    Featured topics include asthma, rhinitis, sinusitis, food allergies, allergic skin diseases, diagnostic techniques, allergens, and treatment modalities. Published material includes peer-reviewed original research, clinical trials and review articles.

    Articles marked "F" offer free full text for personal noncommercial use only.

    The journal is indexed in Thomson Reuters Web of Science and Science Citation Index Expanded, plus the National Library of Medicine's PubMed service.
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