Behçet's syndrome


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Behcet's Syndrome

 

Definition

A group of symptoms that affect a variety of body systems, including musculoskeletal, 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, 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.

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 system is 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 known prevention for Behcet's syndrome.

Resources

Books

Ruddy, Shaun. Kelley's Textbook of Rheumatology. Philadelphia: W.B. Saunders Company, 2001.
Tierney, Lawrence, et al. Current Medical Diagnosis and Treatment. Los Altos: Lange Medical Publications, 2001.

Periodicals

Okada, A. A. "Drug Therapy in Behcet's Disease." Ocular Immunology and Inflammation June 2000: 85-91.
Shed, L. P. "Thalomide Responsiveness in an Infant with Behcet's Syndrome." Pediatrics June 1999: 1295-1297.

Organizations

American Behcet's Disease Association. P.O. Box 280240, Memphis, TN 38168-0240. http://www.behcets.com.
Behcet's Organization Worldwide, Head Office. P.O. Box 27, Watchet, Somerset TA23 OYJ, United Kingdom. http://www.behcets.org.
National Eye Institute. National Institute of Health. Bldg. 31, Rm. 6A32, Bethesda, MD 30892-2510. (800) 869-2020. 2020@nei.nih.gov. http://www.nei.nih.gov.
National Organization for Rare Disorders. P.O. Box 8923, New Fairfield, CT 06812-8923. (800) 999-6673. 〈http://www.rarediseaes.org〉

Key terms

Remission — When active symptoms of a chronic disease are absent.
Uveitis — Inflammation of the area of the eye around the pupil.

Behçet's syndrome (disease)

 [bĕ´chets]
severe uveitis and retinal vasculitis, optic atrophy, and small, shallow, painful, white or gray lesions of the mouth and genitalia, often with other signs and symptoms suggestive of a diffuse vasculitis; it most often affects young males.

Behçet's syndrome

or

Behçet's disease

(bĕch′ĕts′, bĕ-chĕts′)
n.
A type of vasculitis characterized by genital and oral ulcerations and uveitis or iridocyclitis, often accompanied by arthritis.

Behçet's syndrome

A persistent (chronic) disease of obscure origin, causing painful, recurrent ulcers of the mouth and genital area, internal inflammation of the eyes (UVEITIS), ARTHRITIS and blisters, pustules and inflammatory bumps on the skin. (Halushi Behçet, 1889–1948, Turkish dermatologist).