Emergency: requires immediate attention
Angioedema - External and Internal Eye
See also in: Overview,Cellulitis DDx,Oral Mucosal LesionAlerts and Notices
Synopsis
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 swelling being the only manifestation. Angiotensin-converting enzyme (ACE) inhibitors, widely used antihypertensive medications, are a common cause, with angioedema occurring even a year after therapy was initiated. Americans of African descent have 4-5 times greater incidence of ACE inhibitor-induced angioedema than Americans of Northern European descent. ACE inhibitor-induced angioedema often involves the upper airways and can produce a life-threatening risk of respiratory compromise. Other risk factors include female sex, atopy, and cigarette smoking. Other implicated medications include aspirin and other NSAIDs, antibiotics, radiocontrast agents, fibrinolytic agents, and estrogens, including oral contraceptives.
Angioedema as seen in the heritable angioedema syndrome is not responsive to standard antihistamine therapy and usually will not have associated urticaria. 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.
Acquired C1 esterase inhibitor deficiency also presents with angioedema. It is typically the result of a lymphoproliferative disorder (type 1) or autoimmune disease (type 2). It 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.
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 (see alpha-gal syndrome). It is thought that the allergy is mediated by induced immunoglobulin E (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.
Idiopathic angioedema is 3 or more episodes of recurrent angioedema with no apparent cause after comprehensive medical evaluation.
Codes
ICD10CM:T78.3XXA – Angioneurotic edema, initial encounter
SNOMEDCT:
41291007 – Angioedema
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Preseptal cellulitis
- Orbital cellulitis – May present with a facial (eyelid) swelling of rapid onset, but this is associated with signs of an infection, and a cause is readily identified such as an infected tooth or penetrating injury. There are also accompanying fever and leukocytosis, and the patient appears systemically ill. Orbital or preseptal cellulitis is usually unilateral.
- Cavernous sinus thrombosis
- Granulomatous cheilitis – This causes diffuse swelling and is episodic only in its early stages. When well developed, it produces a rubbery, diffuse swelling of the lips. The biopsy reveals the presence of granulomas.
- Serum sickness / serum sickness-like drug eruption
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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.Subscription Required
References
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Last Reviewed:10/31/2019
Last Updated:03/29/2022
Last Updated:03/29/2022
Emergency: requires immediate attention
Patient Information for Angioedema - External and Internal Eye
Overview
Angioedema is the name for swelling of the skin or just under the skin. The most common body locations for this swelling are the lips, eyes and eyelids, and other parts of the face. Less commonly, other parts of the body can be targeted by angioedema, such as the hands, feet, genitals, and throat.Angioedema can be caused by many things, but it is usually an allergic reaction to medication, food, or insect bites and stings.
Who’s At Risk
Angioedema is common. About 10%-20% of the United States' population experiences an angioedema episode at some point in their lives.You may be at increased risk for angioedema if you have a family history or personal history of food allergies, hives, or other allergic reactions.
Angioedema can affect people of any age, but episodes may increase in frequency after the teenage years. Angioedema caused by food allergies are more common in young children.
Signs & Symptoms
The swelling of angioedema may come with pain. The swollen area will be red and warm. Angioedema may or may not be itchy.Self-Care Guidelines
You will want to figure out what is causing your allergic reaction so you can avoid it. Keeping track of your symptoms can help you find out what triggers your angioedema. If you suspect food, keep a food diary.Use over-the-counter antihistamines like Benadryl if your angioedema is not an emergency.
When to Seek Medical Care
Angioedema is usually harmless, but swelling of the throat or tongue may block your airway. If your throat or tongue is swollen, get emergency medical help.Treatments
Antihistamines are the most common treatment for angioedema. Antihistamines reduce both swelling and itching.Corticosteroids such as prednisone reduce swelling in severe cases.
If your angioedema reoccurs often, your doctor may recommend a prescription for anti-inflammatory medication.
Life-threatening angioedema reactions are treated in the emergency room with epinephrine, a type of adrenaline. If this happens to you, your doctor may ask you to carry an "epi-pen" to inject epinephrine in an emergency.
Your doctor may discuss an allergy skin test with you to pinpoint what causes your allergic reaction.
Medications such as blood pressure medications and oral contraceptives can cause angioedema reactions. Tell your doctor what medications you take. You may need to stop taking a medication or find a replacement.
Emergency: requires immediate attention
Angioedema - External and Internal Eye
See also in: Overview,Cellulitis DDx,Oral Mucosal Lesion