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Hereditary Angioedema Differential Diagnoses

  • Author: Michael M Frank, MD; Chief Editor: Michael A Kaliner, MD  more...
 
Updated: Jul 28, 2016
 
 

Diagnostic Considerations

Other types of angioedema without urticaria, referred to as acquired angioedema (AAE), can complicate the diagnostic evaluation of patients with possible hereditary angioedema (HAE). In some cases, AAE may represent a response to an allergen and is treated like urticaria. In other cases, the pathophysiology may be similar to HAE.

As with patients with HAE, those with this type of AAE have decreased C1 inhibitor (C1-INH) activity and experience the same spectrum of clinical disease. However, the 2 conditions differ in several respects. Acquired angioedema is due to (1) an autoantibody to the C1-INH that prevents its function, (2) marked utilization of the normal C1 inhibitor by high levels of antigen-antibody complexes, or (3) factors formed by lymphoid tumors that destroy C1-INH activity.

In general, patients with AAE have low levels of C1q. This distinguishes them from patients with HAE, who have normal levels of the protein.

Although HAE is a treatable disease, the agents used to treat more common forms of angioedema are not effective. Thus, the diagnosis of HAE should be considered when angioedema is not associated with urticaria or when cutaneous or laryngeal attacks do not respond to the usual therapy. Once the diagnosis has been made, efforts should be made to prevent attacks associated with dental and surgical procedures.

Usually, the swelling of HAE does not resemble that of rheumatic disease. Occasionally, when the swelling surrounds a joint and movement of the joint becomes difficult, the difference between HAE and rheumatic disease becomes more difficult to distinguish.

Other problems to be considered in the evaluation of patients with possible HAE are ACE inhibitor–induced angioedema, episodic angioedema with eosinophilia, and vibratory- or pressure-induced angioedema.

Differential Diagnoses

 
 
Contributor Information and Disclosures
Author

Michael M Frank, MD Samuel L Katz Professor of Pediatrics, Professor of Medicine and Immunology, Duke University School of Medicine, Duke University Medical Center

Michael M Frank, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Society for Pediatric Research

Disclosure: Received consulting fee from Shire for consulting; Received honoraria from Robert Michael Educationsl Institute for speaking and teaching; Received consulting fee from BioCryst for consulting.

Chief Editor

Michael A Kaliner, MD Clinical Professor of Medicine, George Washington University School of Medicine; Medical Director, Institute for Asthma and Allergy

Michael A Kaliner, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Clinical Investigation, American Thoracic Society, Association of American Physicians

Disclosure: Nothing to disclose.

Acknowledgements

Dirk M Elston, MD Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Paul Krusinski, MD Director of Dermatology, Fletcher Allen Health Care; Professor, Department of Internal Medicine, University of Vermont College of Medicine

Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Amanda T Moon, MD Resident Physician, Department of Dermatology, University of Rochester, Strong Memorial Hospital

Amanda T Moon, MD, is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Medical Student Association/Foundation, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Kathleen M Rossy, MD Princeton Dermatology Associates

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, New York Academy of Medicine, and Sigma Xi

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

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