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Emergency: requires immediate attention
Angioedema - External and Internal Eye
See also in: Overview,Oral Mucosal Lesion
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Emergency: requires immediate attention

Angioedema - External and Internal Eye

See also in: Overview,Oral Mucosal Lesion
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Contributors: Brandon D. Ayres MD, Christopher Rapuano MD, Harvey A. Brown MD, Sunir J. Garg MD, Lauren Patty Daskivich MD, MSHS
Other Resources UpToDate PubMed

Synopsis

Angioedema (also called angioneurotic edema) is a type 1 hypersensitivity reaction involving tissue edema caused by vasoactive tissue mediators, histamine, kinins, and serotonin. It is a variant of urticaria, with edema occurring at a deeper level, usually within the dermis and subcutis.

Angioedema can be caused by medications, foods, or be idiopathic. Drug-induced angioedema can be associated with urticaria, but it can occur alone with deeper tissue swellings being the only manifestation. The subcutaneous tissues, airway, and gastrointestinal tract are all possible sites of involvement. Angiotensin-converting enzyme (ACE) inhibitors are a common cause, with angioedema occurring even months after therapy has been started. Other causes include aspirin and other non-steroidal anti-inflammatory drugs (NSAIDs), antibiotics, radiocontrast, fibrinolytic agents, and estrogens including oral contraceptives.

Tick bites from some Amblyomma and Ixodes (and possibly Haemaphysalis) species have been associated with the subsequent development of allergies to mammalian meat (eg, beef, pork) in a small number of patients. It is thought that the allergy is mediated by induced IgE antibodies to alpha-gal (galactose-alpha-1,3-galactose), a mammalian oligosaccharide. Individuals with elevated IgE titers to alpha-gal have experienced urticaria, angioedema, and anaphylaxis symptoms either immediately or 3-6 hours (delayed onset) after ingesting mammalian meat (alpha-gal syndrome). Exactly how the tick bite leads to development of this allergy is unclear. Implicated tick bites have been noted to itch for 2 or more weeks. A blood test for these IgE antibodies exists.

Idiopathic angioedema is three or more episodes of recurrent angioedema with no apparent cause after comprehensive medical evaluation.

Non-histaminergic angioedema, which occurs in about 1 of 20 cases, does not present with hives, unlike allergic and idiopathic forms of angioedema. Similar to hereditary angioedema and to ACE inhibitor-induced angioedema, where there is bradykinin involvement, non-histaminergic angioedema is not responsive to antihistamines or corticosteroids.
 
Angioedema seen in the heritable angioedema syndrome lasts longer, is not responsive to standard antihistamine therapy, and usually will not have urticarial lesions. Familial forms begin in adolescence; they have autosomal dominant inheritance and are related to disorders of complement regulation. Episodes of angioedema are often precipitated by surgery or accidents. There are symptoms, such as abdominal pain, that are not a feature of typical angioedema.

Acquired angioedema is typically the result of a lymphoproliferative disorder or autoimmune, neoplastic, or infectious disease. Acquired C1 esterase inhibitor deficiency may result from the formation of autoantibodies against C1 esterase inhibitor or persistent low-level activation of C1q by anti-idiotypic antibodies in patients with B-cell lymphoproliferative disorders. Symptoms of acquired angioedema closely resemble those of hereditary angioedema.

The swelling of angioedema is often more marked than that of cellulitis. Conversely, erythema is a less prominent feature. Unlike cellulitis, angioedema almost always occurs bilaterally. In angioedema, there may also be a history of exposure to a known inciting factor.

Codes

ICD10CM:
T78.3XXA – Angioneurotic edema, initial encounter

SNOMEDCT:
41291007 – Angioedema

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 10/16/2017
Copyright © 2018 VisualDx®. All rights reserved.
Emergency: requires immediate attention
Angioedema - External and Internal Eye
See also in: Overview,Oral Mucosal Lesion
Print 5 Images
View all Images (5)
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Angioedema : Facial edema, Eyelid edema, Eyelids, Lips, Male genital, Skin warm to touch, Tongue edema, Uvula edema, Lip swelling
Clinical image of Angioedema
Copyright © 2018 VisualDx®. All rights reserved.