Behçet's syndrome is chronic inflammation that can cause painful mouth sores, skin blisters, genital sores, and swollen joints. The eyes, blood vessels, nervous system, and digestive tract may also become inflamed.
Behçet's syndrome occurs worldwide but is most common in the area along the silk route from the Mediterranean to China. It is uncommon in the United States. It occurs nearly equally in men and women, typically beginning during their 20s. But it can develop at any age. The cause is unknown.
Almost everyone with this syndrome has recurring, painful mouth sores, similar to canker sores. Sores may appear on the tongue, gums, and lining of the mouth. Sores may also appear on the genital organs. Those on the penis, scrotum, or vulva tend to be painful. Those in the vagina may be painless.
Other symptoms appear days to years later:
Symptoms can come and go unpredictably, becoming very disruptive. Symptoms or symptom-free periods (remissions) may last weeks, years, or decades. Many people eventually go into remission. Occasionally, damage to the nervous system, digestive tract, or blood vessels is fatal.
The diagnosis is based on symptoms and results of a physical examination. No laboratory tests can confirm Behçet's syndrome. Doctors suspect the disorder in people, particularly young adults, who have the following:
However, symptoms may resemble those of many other disorders, including reactive arthritis (previously called Reiter's syndrome), lupus (systemic lupus erythematosus), Crohn's disease, herpes, and ulcerative colitis. The diagnosis may take months to make because doctors look for a pattern of symptoms that subside and recur (remissions and relapses) to help identify the syndrome.
Blood and urine tests are done. They cannot identify the syndrome but can confirm that inflammation is present.
There is no cure, but treatment can usually relieve specific symptoms. Which drugs are used depends on which organ is affected and how severe the disease is. For example, the following may be used:
Azathioprine may also reduce the number of mouth and genital sores, help sores heal, and reduce joint pain. Cyclophosphamide and chlorambucil are used when other drugs are ineffective or when life-threatening complications develop.
Last full review/revision April 2008 by Carmen E. Gota, MD