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Emergency: requires immediate attention
Angioedema in Infant/Neonate
See also in: External and Internal Eye,Oral Mucosal Lesion
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Emergency: requires immediate attention

Angioedema in Infant/Neonate

See also in: External and Internal Eye,Oral Mucosal Lesion
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Contributors: Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Lynn Garfunkel MD, Nancy Esterly MD
Other Resources UpToDate PubMed

Synopsis

Angioedema is an allergy-mediated tissue edema characterized by transient dermal swelling. It is a variant of urticaria, with the edema occurring at a deeper level, usually within the dermis and subcutis. Angioedema can be caused by certain medications (antibiotics, cardiac drugs, immunotherapeutics, chemotherapeutics, angiotensin-converting enzyme [ACE] inhibitors, calcium channel blockers, and histamine releasers), foods (eg, milk, eggs, shellfish, wheat, and nuts), or it may be idiopathic.

There are 3 mechanisms thought to be responsible:
  • IgE mediated – IgE sensitizes mast cells to release vasoactive substance. Seen with reaction to antibiotics or radiocontrast.
  • Complement mediated – The activated complement causes anaphylotoxin release, which causes mast cell degranulation. Seen with whole blood or immunoglobulin infusions.
  • Immune complex mediated – Immune complexes cause mast cell degranulation. Seen in 1/10 000 courses of penicillin.
Drug-induced angioedema can be associated with urticaria but can occur alone, with deeper tissue swellings being the only manifestation. The subcutaneous tissues, airway, and gastrointestinal tract are all possible sites of involvement. ACE inhibitors are a common cause, with angioedema occurring even months after therapy has been started. Other causes include aspirin and other 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. 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 3 or more episodes of recurrent angioedema with no apparent cause after comprehensive medical evaluation.

Nonhistaminergic 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, nonhistaminergic 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 typical 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.

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: 06/15/2018
Copyright © 2019 VisualDx®. All rights reserved.
Emergency: requires immediate attention
Angioedema in Infant/Neonate
See also in: External and Internal Eye,Oral Mucosal Lesion
Print 2 Images
View all Images (2)
(with subscription)
Angioedema : Facial edema, Eyelid edema, Eyelids, Lips, Male genital, Skin warm to touch, Tongue edema, Uvula edema, Lip swelling
Clinical image of Angioedema
Copyright © 2019 VisualDx®. All rights reserved.