Behcets disease is a rare, chronic disorder involving inflammation of blood vessels throughout the body. It is marked by recurrent oral and genital ulcers and eye inflammation.
The cause of Behcets disease is unknown. It is believed to be caused by an autoimmune reaction. This occurs when the body’s immune system attacks its own tissue by mistake. This autoimmune abnormality may be inherited. An environmental trigger, such as a specific virus or bacterium, may activate the disease in people susceptible to it.
Although the exact cause of Behcets disease is unknown, some groups of people are more likely to develop the condition than others. Factors that may increase your risk of Behcets include:
- Location: the Middle East, Asia, and Japan
- Family history of Behcets disease
- A gene marker called the HLA-B51/B5 allele
- In the United States, men are more likely than women to develop this condition.
- In the Middle East, Asia, and Japan, women are more likely than men to develop Behcets.
- Age: 20's and 30's
Symptoms of Behcets disease can vary from mild to very severe. Symptoms tend to appear, heal, and then recur (referred to as a flare) frequently over months or years. The most common symptoms of the disease are:
Other symptoms of the disease may include:
Diagnosing Behcets disease is very difficult because:
- Symptoms are similar to those of many other diseases.
- Symptoms often appear very slowly, over months or years.
- There is no specific test to confirm the disease.
A doctor may suspect Behcets disease if oral sores appear at least three times within a year and at least two of the following recurring symptoms appear:
- Eye inflammation
- Genital sores
- Skin sores
If Behcets disease is suspected, your doctor will do tests to rule out other diseases with similar symptoms. In addition, you may have a pathergy skin test. For this test, your skin is pricked with a small needle. If you have Behcets disease, a bump will develop at the site of the skin prick. However, this test is not conclusive. Many people with the disease do not have a reaction to the skin prick.
There is no cure for Behcets disease however, spontaneous regression may occur. Treatment is aimed at limiting and preventing complications from symptoms. With proper treatment, the disease can usually be managed fairly well. Treatment includes medications, rest, and exercise.
Both topical and oral medications are used.
Topical medications include creams, lotions, and mouth rinses that contain corticosteroids (to cut down on inflammation) and/or anesthetics (to decrease pain). These are applied to sores to reduce inflammation and pain.
Oral medications include:
- Oral corticosteroids—to reduce inflammation and pain
Immunosuppressive drugs—to help control the overactive immune system. These may include:
Many of the drugs used to treat Behcets disease can cause severe side effects. Your doctor must closely monitor you if you are taking these medications
General guidelines include:
- Rest when symptoms flare to help speed healing.
- Engage in moderate exercise when symptoms recede to help keep joints flexible and strong.
There are no guidelines for the prevention of Behcets disease because the exact cause is unknown.
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Last reviewed August 2013 by Michael Woods MD
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