Information on Behcet Syndrome

Behcet's syndrome is a disease that involves inflammation of the blood vessels. It causes problems in many parts of the body. Behcet's syndrome is classically characterized as a triad of symptoms that include recurring crops of mouth ulcers (aphthous ulcers), genital ulcers, and inflammation of a specialized area around the pupil of the eye (the uvea). The inflammation of the area of the eye that is around the pupil is called uveitis. Behcet's syndrome is also sometimes referred to as Behcet's disease.

Behçet disease (Behçet's syndrome, Morbus Behçet, silk road disease) is a chronic condition due to disturbances in the body’s immune system. This system, which normally protects the body against infections through controlled inflammation, becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects blood vessels, usually the small ones. As a result, symptoms occur wherever there is a patch of inflammation, and can be anywhere where there is a blood supply.

The cause of Behcet's syndrome is unknown. Symptoms include recurring ulcers in the mouth or the genital area, skin lesions, arthritis that affects mainly the knees and ankles, pain and irritation in the eyes, and fever. The mouth and genital ulcers tend to occur in multiples and can be quite painful.

In the mouth, these ulcers are generally found on the tongue, gums, and the inside of the lips or jaws. In the genital area, the ulcers usually occur on the penis and scrotum in males and on the vulva of women. The eye inflammation can lead to blindness.

Onset of the disease is usually between the ages of 20 and 30, although some patients have been as young as 4 and as old as 70. Three cases of Behçet's disease were described among newborns born to mothers with disease. The transmission of the disease from mother to newborn might be caused by a specific antibody entering the fetus through the placenta.

The literature suggests that the clinical picture of Behçet's syndrome in children differs from that in adults, in that there is a lower frequency of eye disease and unusual manifestations appear to be more common. Behçet's disease affects twice as many men as women.

Most of the symptoms are painful but not life-threatening. They come and go in a series of attacks (‘flare-ups’) throughout life. The disease does not ‘burn itself out’, but sometimes it can level off and the flare-ups become less aggressive and happen less frequently. Most people with Behçet’s disease have a normal lifespan and can hope to lead close-to-normal daily lives.

Behçet syndrome is characterized by recurrent aphthous ulcers, genital ulcers, and uveitis or retinal vasculitis. Other manifestations of the disease include skin lesions, arthritis, gastrointestinal lesions, central nervous system (CNS) involvement, and vascular lesions, including aneurysms and thrombosis.

In Behçet syndrome, the basic lesion is vasculitis. Biopsies have shown vasculitis near lesions of Behçet syndrome, including the oral and genital ulcers and lesions of the CNS and the eyes; large vessels are affected by a vasculitis of the vasa vasorum. Vascular injuries may be superimposed on the hypercoagulability observed in some patients.

Treatment of Behçet’s disease is symptomatic and empirical. Arthritis responds to analgesics and rest. Mucous membrane involvement may respond to topical corticosteroids in the form of paste or mouthwash. The therapy of CNS lesions usually entails high dose glucocorticosteroids and immunosuppressants. Gastrointestinal involvement may be managed with sulfasalazine.