Behcet's Syndrome

What Is It?

Behçet's (bay-setz) disease, also called Behçet's syndrome, is a rare disease that mainly affects young adults. Named after Hulusi Behçet, a Turkish dermatologist, it involves the inflammation of many parts of the body, including the skin of the genital area, lining of the mouth, eye, nervous system, joints and blood vessels. The most characteristic problems include ulcers in the mouth and genital areas, and serious eye inflammation.

Although the exact cause of the disease remains unclear, Behçet's is thought to involve an autoimmune response, which means the body's defense mechanism malfunctions and begins to attack its own tissues. Researchers believe that something in the environment may trigger the symptoms in people susceptible to autoimmune problems. For example, some studies report that people who have had repeated infections with Streptococcus bacteria are more likely to have Behçet's disease.

Usually, symptoms flare up repeatedly and then disappear. The time between attacks tends to be unpredictable — as short as a few days or as long as years. However, in some people with more severe disease, symptoms vary in intensity but are always present.

On average, the first symptoms appear between ages 20 and 30. It affects up to one in 5,000 people in some populations (such as in Japan and Saudi Arabia), but, for unknown reasons, as few as one in 500,000 in other groups (including North Americans and Northern Europeans).

Symptoms

Symptoms are different in each person. Some have only mild symptoms, while others have more severe ones.

People with Behçet's disease usually have painful, yellowish sores, similar to canker sores, in the mouth. While canker sores generally occur one at a time, people with Behçet's disease tend to have multiple sores. These usually go away within two weeks, but often return. More than half of people with Behçet's have sores around the genitals that resemble the oral sores. Behçet's can cause rashes, including acne-like bumps on the skin and inflammation around hair follicles. Blood clots can occur, usually in the legs, and can cause pain and tenderness.

Many people with Behçet's disease have joint pain and swelling, usually in the knees, ankles and wrists. Although the joints can be stiff and painful for several weeks at a time, the disease usually does not cause long-term joint damage or deformity.

Up to 75 percent of people with Behçet's disease have eye inflammation, particularly a condition called uveitis that usually shows up within two years of the first symptoms. Uveitis is more common in men than in women. At first, it may not cause any pain or vision problems, although it can cause blurred vision. Repeated flare-ups of uveitis can lead to decreased vision or blindness.