by McCoy K
(Angioneurotic Edema; Hereditary Angioedema)

Definition

Angioedema is swelling under the skin with or without redness. It can happen around the eyelids, lips, on the face, hands, feet, or groin. It often shows up in an uneven pattern. Angioedema is similar to hives but affects deeper layers of tissue.

Angioedema can cause airways to swell. It is important to seek medical care for it.

Causes

Angioedema is often caused by the body's abnormal reaction to a trigger. It is not clear why this happens. Common triggers include:

  • Foods such as fruits, shellfish, and nuts
  • Medicine such as:
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Angiotensin-converting enzyme (ACE) inhibitors
  • Angiotensin receptor blockers (ARB) inhibitors
  • Penicillin
  • Aspirin
  • Morphine
  • Infections
  • Inhaling things like pollens, mold spores, and animal dander
  • Certain diseases such as hyperthyroidism, cancer, and rheumatic fever
  • Cold or hot environments
  • Touching plants, animals, or some medicine
  • Skin disease

Hereditary or nonhistaminergic angioedema (HAE) is caused by changes in genes. This leads to abnormal levels of certain proteins that control swelling. HAE is rare and can be passed through genes from parents to children.

It is not always known why a person gets angioedema.

Hives
Splotchy body rash -adult
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Risk Factors

It is more common in women and people aged 30 to 60 years. Other things that may increase the risk of it are:

  • Having health issues such as:
  • Drinking alcohol
  • Taking NSAIDs
  • Feeling a lot of worry and stress
  • Hyperthyroidism
  • Having a family history of it

Symptoms

A person with angioedema may have:

  • Large patches of swelling around the eyelids and lips
  • Sores or spots on the body
  • Swelling of the throat, hands or feet
  • Belly pain
  • A rash that is not itchy

Diagnosis

The doctor will ask about symptoms and past health. A physical exam will be done. The doctor may ask about foods, medicines, or other triggers. They may also ask about family health history.

The diagnosis can be made by sight. Tests may be done if causes are not clear and it happens often.

Treatment

Minor angioedema may not need to be treated. It can help to find and avoid triggers when possible.

Swelling in or near an airway needs immediate medical care.

Treatment steps for any angioedema may include:

  • Antihistamines—can ease swelling and itching.
  • Corticosteroid drugs—to ease swelling.
  • Epinephrine—fast-acting medicine for severe reactions. This medicine can be carried for emergency use.

HAE is treated with specific medicine. It will help to balance proteins that manage swelling. This should stop or reduce abnormal swelling.

Very bad swelling can close off the airway. Medicine may not be able to control it in time. A tracheostomymay be needed. A small opening is made in the neck below the swelling. This will allow air to get in and out of the lungs until the swelling goes away.

Tracheostomy Tube
Tracheostomy Tube
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Prevention

It is not clear why some people develop angioedema. There are no steps to prevent it. Knowing and avoiding triggers can help to manage it.

RESOURCES

American Academy of Allergy, Asthma & Immunology  http://www.aaaai.org 

Asthma and Allergy Foundation of America  http://www.aafa.org 

CANADIAN RESOURCES

Canadian Dermatology Association  https://dermatology.ca 

Canadian Society of Allergy and Clinical Immunology  http://csaci.ca 

References

Acute urticaria. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/chronic-urticaria. Accessed May 12, 2022.

Angioedema. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/angioedema. Accessed May 12, 2022.

Hereditary angioedema. DermNet NZ website. Available at: https://dermnetnz.org/topics/hereditary-angioedema. Accessed May 13, 2022.

Tarbox J, Bansal A, Peiris A. Angioedema. JAMA, 2018; 319(19): 2054.

Treatment options. U.S. Hereditary Angioedema Association website. Available at: https://www.haea.org/pages/p/treatment. Accessed May 12, 2022.

Revision Information

  • Reviewer: EBSCO Medical Review Board Marcin Chwistek, MD
  • Review Date: 03/2022
  • Update Date: 05/13/2022