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Harvard Medical School

Behcet's Syndrome

What Is It?

Behçet's (bay-setz) syndrome, also called Behçet's disease, 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. A genetic susceptibility is suggested by the occasional finding of this rare disease in multiple members of the same family.

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 1 in 5,000 people in some populations (such as in Japan and Saudi Arabia), but, for unknown reasons, as few as 1 in 500,000 in other groups (including North Americans and Northern Europeans).