Angioedema is swelling of areas of tissue under the skin, sometimes affecting the face and throat.
Angioedema can be a reaction to a medication or other substance (trigger), a hereditary disorder, a rare complication of cancer, or an immune disorder, but sometimes the cause is not known.
Angioedema may involve swelling in the face, throat, digestive tract, and airways.
Antihistamines can relieve mild symptoms, but if angioedema makes swallowing or breathing difficult, prompt emergency treatment is needed.
(See also Overview of Allergic Reactions.)
Angioedema often occurs with hives, which are small, itchy, slightly elevated areas of swelling that may be red or similar to the tone of the person's skin, often with a pale center. Both hives and angioedema involve swelling, but in angioedema, the swelling is deeper (under the skin) than it is with hives, and it may not itch.
There are rare hereditary or acquired forms of angioedema. These forms are caused by a deficiency or malfunction of a substance called C1 inhibitor, which is part of the immune system. Hereditary angioedema and acquired angioedema resemble angioedema caused by an allergic reaction. However, hives do not develop, and the cause is different.
Angioedema may be acute or chronic.
Acute angioedema
Acute angioedema develops suddenly after exposure to a trigger.
Common triggers are
Insect stings or bites
Allergy injections (allergen immunotherapy)
Certain foods—particularly eggs, fish, shellfish, nuts, and fruits
Angioedema without hives is sometimes caused by ACE inhibitors, which are used to treat high blood pressure and heart failure. When ACE inhibitors are the cause, the face and upper airways are most commonly affected, but the intestine may be affected.
Eating even a tiny amount of some foods can suddenly result in angioedema. But with other foods (such as strawberries), these reactions occur only after a large amount is eaten.
Also, angioedema sometimes occurs after a substance that can be seen on x-rays (radiopaque contrast agent) is used during an imaging test.
Chronic angioedema
Angioedema can be chronic, recurring over weeks or months. The cause is usually unknown, but it may be repeated, unintentional intake of a substance, such as penicillin in milk or a preservative or dye in foods. Use of certain medications, such as aspirin, other NSAIDs, or opioids, can also cause chronic angioedema.
Chronic angioedema that occurs without hives may be hereditary or acquired angioedema.
However, usually when angioedema without hives recurs, no specific cause is identified. Angioedema with no identified cause is called idiopathic angioedema.
Symptoms of Angioedema
In angioedema, swelling may not be equal on both sides of the body, and it can be mildly painful. It can affect the back of the hands or feet, eyelids, tongue, face, lips, or genitals. Sometimes the membranes lining the mouth, throat, and airways swell, making swallowing or breathing difficult.
Occasionally, the digestive tract is involved, resulting in nausea, vomiting, crampy abdominal pain, or diarrhea.
Many people also have hives. Hives usually begin with itching. Typically, crops of hives then come and go. One spot may remain for several hours, then disappear, and later, another may appear elsewhere. After the hive disappears, the skin usually looks completely normal.
People may have an anaphylactic reaction (a sudden, widespread, sometimes life-threatening allergic reaction). Blood pressure may become dangerously low (shock).
This photo shows swelling of the lips in a person with hereditary angioedema.
By permission of the publisher. From Joe E, Soter N. In Current Dermatologic Diagnosis and Treatment, edited by I Freedberg, IM Freedberg, and MR Sanchez. Philadelphia, Current Medicine, 2001.
This person has a swollen tongue due to angioedema.
SCIENCE PHOTO LIBRARY
This person has swollen lips due to angioedema.
DR P. MARAZZI/SCIENCE PHOTO LIBRARY
This photo shows swelling of the lips in a person with hereditary angioedema.
By permission of the publisher. From Joe E, Soter N. In Current Dermatologic Diagnosis and Treatment, edited by I Freedberg, IM Freedberg, and MR Sanchez. Philadelphia, Current Medicine, 2001.
This person has a swollen tongue due to angioedema.
SCIENCE PHOTO LIBRARY
This person has swollen lips due to angioedema.
DR P. MARAZZI/SCIENCE PHOTO LIBRARY
Diagnosis of Angioedema
A doctor's evaluation
People should see a doctor immediately if they develop angioedema.
The cause of angioedema is often obvious, and tests are seldom needed because the reactions usually resolve and do not recur.
If angioedema recurs and the cause is not clear, doctors ask people about all the medications they are taking and all food and drink they are consuming. If the cause is still not clear, particularly if people have no hives or if family members have angioedema, doctors may do tests for hereditary or acquired forms of angioedema.
Treatment of Angioedema
Antihistamines
Corticosteroids
If the cause of angioedema is not obvious, all nonessential medications should be stopped until symptoms resolve.
For mild angioedema with hives, taking antihistamines partially relieves the itching and reduces the swelling. Corticosteroids, taken by mouth, are prescribed for severe symptoms when other treatments are ineffective, and they are given for as short a time as possible. When taken by mouth for more than 3 to 4 weeks, they have many, sometimes serious side effects. Corticosteroid skin preparations (such as creams, ointments, and lotions) do not help.
For angioedema without hivesfresh frozen plasma
For idiopathic angioedema, a high dose of an antihistamine, taken by mouth, may help.
Emergency treatment
If severe angioedema results in difficulty swallowing or breathing or in collapse, prompt emergency treatment is necessary. Doctors must then open the person's airway by doing one or both of the following:
Inserting a breathing tube through the person’s mouth or nose and into the windpipe (intubation)
If the airway closes too fast, doctors may have to make a small incision in the skin over the windpipe and insert a breathing tube.
After a severe allergic reaction, such people should go to the hospital emergency department, where they can be checked and treated as needed.
