Behcet's syndrome

Definition

A group of symptoms that affect a variety of body systems, includingmusculoskeletal, gastrointestinal, and the central nervous system. These symptoms include ulceration of the mouth or the genital area, skin lesions, and inflammation of the uvea (an area around the pupil of the eye).

Description

Behcet's syndrome is a chronic disease that involves multiple body systems.


The disease is named for a Turkish dermatologist, Hulusi Behcet, who first reported a patient with recurrent mouth and genital ulcers along with uveitis in 1937. The disease occurs worldwide, but is most prevalent in Japan, the Middle East, and in the Mediterranean region. There is a wider prevalence among males than females in a ratio of two to one.



Causes and symptoms

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, andfever. 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 inmales and on the vulva of women. The eye inflammation can lead to blindness.

Diagnosis

Because Behcet's syndrome is a multisystem disease, it is difficult to diagnose. International criteria have been proposed to assist in classifying this disease. There is no one diagnostic feature of this disease, so diagnosis depends on grouping together enough symptoms in order to identify the disease. Symptoms of Behcet's syndrome also occur in other diseases, so it is often necessary to rule out the other diseases before a definitive diagnosis can be reached.

Treatment

Some of the current drugs used to treat Behcet's syndrome include corticosteroids, cyclosporine, azathioprine, chlorambucil, interferon alpha, thalidomide, levamisole, and pulse cyclophosphamide.

Prognosis

The prognosis for Behcet's syndrome is generally poor. There has been a documented case of Behcet's lasting for 17 years. Although the disease is considered painful but not fatal, when the central nervous systemis involved there is usually severe disability and death often occurs. The condition is usually chronic, although there can be remissions during the course of the disease. There is no predictable method to determine which patients will progress into the more serious symptoms, and which might move into remission.

Prevention

There is no knownprevention forBehcet's syndrome.