Behcet's Syndrome

What Is It?

Behçet's (bay-setz) syndrome, also called Behçet's disease, is a rare disease that mainly affects young adults. Named after Hulusi Behçet, a Turkish dermatologist, it involves the inflammation of many parts of the body, including the skin of the genital area, lining of the mouth, eye, nervous system, joints and blood vessels. The most characteristic problems include ulcers in the mouth and genital areas and serious eye inflammation.

Although the exact cause of the disease remains unclear, Behçet's is thought to involve an autoimmune response, which means the body's defense mechanism malfunctions and begins to attack its own tissues. Researchers believe that something in the environment may trigger the symptoms in people susceptible to autoimmune problems. For example, some studies report that people who have had repeated infections with Streptococcus bacteria are more likely to have Behçet's disease. A genetic susceptibility is suggested by the occasional finding of this rare disease in multiple members of the same family.

Usually, symptoms flare up repeatedly and then disappear. The time between attacks tends to be unpredictable — as short as a few days or as long as years. However, in some people with more severe disease, symptoms vary in intensity but are always present.

On average, the first symptoms appear between ages 20 and 30. It affects up to one in 5,000 people in some populations (such as in Japan and Saudi Arabia), but, for unknown reasons, as few as one in 500,000 in other groups (including North Americans and Northern Europeans).

Symptoms

Symptoms are different in each person. Some have only mild symptoms, while others have more severe ones.

People with Behçet's disease usually have painful, yellowish sores, similar to canker sores, in the mouth. While canker sores generally occur one at a time, people with Behçet's disease tend to have multiple sores. These usually go away within two weeks, but often return. More than half of people with Behçet's have sores around the genitals that look like the mouth sores. Behçet's can cause rashes, including acne-like bumps on the skin and inflammation around hair follicles. Blood clots can occur, usually in the legs, and can cause pain and tenderness.

Many people with Behçet's disease have joint pain and swelling, usually in the knees, ankles and wrists. Although the joints can be stiff and painful for several weeks at a time, the disease usually does not cause long-term joint damage or deformity.

Up to 75% of people with Behçet's disease have eye inflammation, particularly a condition called uveitis that usually shows up within two years of the first symptoms. Uveitis is more common in men than in women. At first, it may not cause any pain or vision problems, although it can cause blurred vision. Repeated flare-ups of uveitis can lead to decreased vision or blindness.

In about 10% of cases, Behçet's disease causes inflammation of the central nervous system (brain and spinal cord). Symptoms include fever, stiff neck, headache and uncoordinated movements.

Rarely, Behçet's will inflame the intestinal tract, causing diarrhea or constipation, vomiting and stomach pain.

Diagnosis

Diagnosing Behçet's can be difficult because it is rare and its symptoms can be similar to those of other diseases. Certain characteristics of this disease can be helpful in diagnosis because they are so rare in other conditions. For example, the combination of oral and genital ulcers that are recurrent, simultaneous and multiple are suggestive of the disease; similarly, inflammation of the lungs' arteries in Behçet's may cause an aneurysm, called pulmonary artery aneurysm which rarely occurs in any other condition.

Some doctors test for Behçet's by pricking the skin with a needle. This needle-prick test is called a pathergy test. One or two days later, people with Behçet's can develop a lump or nodule where the needle broke the skin. If the patient has had blood taken with a needle for another test, a pathergy test is not needed because the site of the blood test can be observed to see if a nodule forms. This test usually is not used in the United States because few people with Behçet's in North America or Northern Europe have a reaction to the needle. However, people in certain other countries, such as Japan, often do.

Although blood tests and a skin biopsy can support the diagnosis, no tests can confirm or rule out Behçet's disease with great certainty. In a biopsy, a small piece of skin is removed to be examined in a laboratory. To diagnose the disease, doctors look for the key symptoms — recurring mouth sores, genital sores, skin rashes and eye inflammation.

Expected Duration

Behçet's disease can be a chronic (long-lasting) condition, although the symptoms sometimes diminish over time and disappear for as long as several years.

Prevention

There is no way to prevent Behçet's disease.

Treatment

Treatment focuses on relieving the symptoms, and depends on which parts of the body the disease affects:

• Rashes and mouth sores:
  • For mild disease Corticosteroids (ointments, creams, rinses) applied to the skin (topical)
  • For more significant disease or if topical treatment is not successful Colchicine (sold as a generic), dapsone (DDS) or thalidomide (Thalomid)
  • For more severe disease Immune suppressants, including a pill form of corticosteroid, azathioprine (Imuran) or methotrexate (Folex, Methotrexate LPF, Rheumatrex)
  • For mouth pain Novocaine-type agents or a mixture of Kaopectate, diphenhydramine (Benadryl) and lidocaine as a mouth rinse can provide at least temporary relief
• Arthritis The most commonly prescribed medications are colchicine, nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, hydroxychloroquine (Plaquenil), sulfasalazine (Azulfidine) and methotrexate
• Eye disease Corticosteroids (drops, injections or pills), colchicine, immunosuppressants, including cyclophosphamide (Cytoxan, Neosar), cyclosporine (Neoral, Sandimmune) or chlorambucil (Leukeran)

Some studies suggest that interferon (Betaseron and others)) or infliximab (Remicade) may improve severe disease that has not responded to other treatment. For severe disease that affects many parts of the body, combinations of medications may be prescribed.

Non-medication therapy for joint pain, including exercise, is encouraged as soon as symptoms allow.

When To Call A Professional

Make an appointment with your doctor if you have unexplained sores in your mouth or other symptoms of Behçet's disease.

Prognosis

Most people with Behçet's disease are able to control their symptoms and live normal lives. If the first symptoms cannot be controlled with medications, more serious symptoms, such as inflammatory eye disease, can appear. Those more likely to develop more severe disease include men, people of Middle Eastern or Asian descent, and people who develop the disease at a younger age.

Additional Info

The American Behçet's Disease Association

PO Box 54063

Minneapolis, MN 55454-0063

Phone: (800) 723-4238

http://www.behcets.com/

American College of Rheumatology

1800 Century Place, Suite 250

Atlanta, GA 30345

Phone: (404) 633-3777

Fax: (404) 633-1870

E-Mail: acr@rheumatology.org

http://www.rheumatology.org/

American Autoimmune Related Diseases Association (AARDA)

22100 Gratiot Ave.

East Detroit, MI 48021

Phone: (586) 776-3900

http://www.aarda.org/

Arthritis Foundation

P.O. Box 7669

Atlanta, GA 30357-0669

Phone: (404) 872-7100

Toll-Free: (800) 283-7800

http://www.arthritis.org/

National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

1 AMS Circle

Bethesda, MD 20892-3675

Phone: (301) 495-4484

Toll-Free: (877) 226-4267

Fax: (301) 718-6366

TTY: (301) 565-2966

E-Mail: niamsinfo@mail.nih.gov

http://www.niams.nih.gov/