Behçet disease is chronic blood vessel inflammation (vasculitis) 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 disease occurs worldwide but is most common in the area along the silk route from the Mediterranean to China. It is relatively uncommon in the United States. It occurs nearly equally in men and women, typically beginning during the 20s, but tends to be more severe in men. Occasionally, the syndrome develops in children. The cause is unknown.
Almost everyone with this syndrome has recurring, painful mouth sores, similar to canker sores. Mouth sores are usually the first symptom of Behçet disease. Sores may appear anywhere in the mouth, such as on the tongue, gums, and lining of the mouth, and often occur in clusters. The sores are round or oval, less than half an inch to about 4 inches (about 1 to 10 centimeters) in diameter, and shallow or deep with a yellowish center. The sores last 1 to 2 weeks. Sores may also appear on the genital organs. Sores on the penis, scrotum, or vulva tend to be painful. Sores in the vagina may be painless. Although other kinds of mouth sores are very common (for example, cold sores caused by the herpes simplex virus), the sores caused by Behçet disease tend to last longer and be more severe.
People may have a fever and a general feeling of illness (malaise). Other symptoms appear days to years later:
The diagnosis is based on symptoms and results of a physical examination. No laboratory tests can confirm Behçet disease. Doctors suspect the disorder in people, particularly young adults, who have had 3 episodes of mouth sores in 1 year and 2 of the following:
However, symptoms may resemble those of many other disorders, including reactive arthritis (previously called Reiter syndrome), lupus (systemic lupus erythematosus), Crohn disease, herpes, and ulcerative colitis. The diagnosis may take months to make because doctors look for a pattern of symptoms that subside (called remission) and recur (called relapse) to help identify the syndrome. Mouth sores can resemble other, more common sores, such as fever blisters (caused by the herpes simplex virus).
Blood and urine tests are done. They cannot identify the syndrome but can confirm that inflammation is present.
The diagnosis is difficult to confirm when people have only mouth sores and possibly sores on their genitals or skin. The diagnosis is much easier to make when other symptoms, such as inflammation of the eyes or blood vessels, are present.
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 risk of death is highest for men and people with disease of the arteries or a high number of flare-ups. With time, the disease seems to quiet down.
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:
Cyclophosphamide and chlorambucil are used when other drugs are ineffective or when life-threatening complications develop.
Last full review/revision April 2013 by Carmen E. Gota, MD