Diagnostic Considerations

Special consideration should be given to those who experience angioedema without urticaria. In such cases, hereditary and acquired angioedema (AAE) must be differentiated.^[14]

When angioedema is associated with urticaria, the diagnostic algorithm is almost identical to that of urticaria patients. For recurrent angioedema without urticaria, it is strongly recommended to rule out hereditary angioedema (HAE), angiotensin-converting enzyme inhibitor induced angioedema (ACEI-AAE, or AIIA), and acquired C1 esterase inhibitor deficiency angioedema (C1-INH-AAE).^[49]

Except for ACEI-AAE (or AIIA), C1-INH-AAE, and different types of HAE, a significant proportion of angioedema can be adequately controlled with daily doses of nonsedating antihistamines.^[14]Therefore, for angioedema without urticaria, and once C1 INH deficiency and ACEI-AAE are ruled out, empirical treatment with high doses of a second generation antihistamine (up to 4 times more than the conventional dose) can help further categorize the patients (histaminergic vs. nonhistaminergic).

Differential Diagnoses

Workup

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Media Gallery

Photographic documentation of swelling.
Bradykinin production and metabolism.
Classification of angioedema without urticaria based on clinical or etiopathologic features. AAE = acquired angioedema; ACEI = angiotensin-converting enzyme inhibitors; HAE = hereditary angioedema; Specific triggers = food, drug, insect bite, environmental allergen, or other physical stimulus. Based on data from Zingale LC, Beltrami L, Zanichelli A, et al. Angioedema without urticaria: a large clinical survey. CMAJ. Oct 24 2006; 175(9): 1065–70.
Angioedema secondary to angiotensin-converting enzyme (ACE) inhibitors.
Types of angioedema.
Pathways for production of prostaglandins and leukotrine from mobilized arachidonic acid.

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Author

Huamin Henry Li, MD, PhD, CPI Director of Chevy Chase Clinical Research, Institute for Asthma and Allergy; Assistant Professor, George Washington University Hospital; Clinical Faculty, Johns Hopkins Asthma and Allergy Center, Johns Hopkins Hospital

Huamin Henry Li, MD, PhD, CPI is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, Joint Council of Allergy, Asthma and Immunology, MedChi The Maryland State Medical Society

Disclosure: Received consulting fee from Dyax for consulting; Received consulting fee from Pharming/Salix for consulting; Received consulting fee from CSL Behring for consulting; Received honoraria from Viropharma/Shire for speaking and teaching; Received consulting fee from Viropharma/Shire for consulting; Received honoraria from Dyax for speaking and teaching.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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

Stephen C Dreskin, MD, PhD Professor of Medicine, Departments of Internal Medicine, Director of Allergy, Asthma, and Immunology Practice, University of Colorado Health Sciences Center

Stephen C Dreskin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association for the Advancement of Science, American Association of Immunologists, American College of Allergy, Asthma and Immunology, Clinical Immunology Society, and Joint Council of Allergy, Asthma and Immunology

Disclosure: Genentech Consulting fee Consulting; American Health Insurance Plans Consulting fee Consulting; Johns Hopkins School of Public Health Consulting fee Consulting; Array BioPharma Consulting fee Consulting

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