Behçet’s syndrome is a systemic vasculitis, most commonly presenting with oral and genital ulceration, skin lesions ranging from acne to erythema nodosum, and eye and central nervous system involvement, which cause most of the serious morbidity associated with the disease.
It starts most in the 3rd decade of life, male: female ratios are similar but the disease is more severe in males, especially young male patients. Symptoms can develop over time and can be thought of as a continuum when making the diagnosis.
The diagnosis is made through clinical signs and symptoms. There are no blood tests or radiographic findings that are specific to Behçet’s syndrome.
Most commonly used classification criteria are that patients need to have oral ulcers and 2 of genital ulcerations, skin lesions, eye involvement and pathergy positivity. There are rare patients where oral ulceration is not present but other symptoms of Behçet’s syndrome are seen. Other diseases that can mimic findings of Behçet’s syndrome have to be worked up and ruled out.
Treatment depends on the severity of symptoms. For mucocutaneous lesions colchicine, low dose steroids, topical creams can be used. When there is eye involvement, or more resistant symptoms, immunosuppressive medications should be used in the treatment. Azathioprine is usually preferred, a TNF inhibitor or cyclosporine can be added for more severe cases.
The natural history of Behçet’s syndrome is one of decreasing disease activity with time. Most patients do better with time and require less medication than initially required to treat the disease. This important aspect of the disease needs to be remembered when treatment decisions are made.
Additional information and resources are available on the American Behçet’s Disease Association website at http://www.behcets.com.
Care for individuals with Behçet’s syndrome is provided at our Behçet's Syndrome Center.