Originally published by Harvard Health.

What Is It?

Behçet’s (bay-setz) syndrome is a rare disease that causes inflammation of many parts of the body. These include 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. It is also called Behçet’s syndrome.

The exact cause of the disease remains unclear. But Behçet’s disease is thought to involve an autoimmune response. This means the body’s defense mechanism begins to attack its own tissues. Something in the environment may trigger this abnormal immune response in susceptible individuals. Genetic factors may also play a role.

Usually, symptoms flare up repeatedly and then improve. The time between attacks may be as short as a few days or as long as years. In some people with more severe disease, symptoms vary in intensity but are always present.

The first symptoms usually appear between ages 20 and 30.

Symptoms

Symptoms are different in each person. They range from mild to severe.

People with Behçet’s disease usually have painful, sores in the mouth that are quite similar or identical to canker sores. But while canker sores generally occur one at a time, people with Behçet’s disease tend to have multiple sores.

More than half of people with Behçet’s disease have sores around the genitals. These sores look like the mouth sores.

Behçet’s disease can cause rashes, including acne-like bumps on the skin. It can cause inflammation around hair follicles. Blood clots can occur, usually in the legs. They can cause pain and tenderness.

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

Up to three-quarters of people with Behçet’s disease have eye inflammation. Specifically, a condition called uveitis usually shows up within two years of the first symptoms. At first, uveitis can cause blurred vision. Repeated flare-ups of uveitis can lead to decreased vision or even blindness.

In about 10% of cases, Behçet’s disease causes inflammation of the brain and spinal cord. Symptoms include fever, stiff neck, headache and uncoordinated movements.

Rarely, Behçet’s disease will inflame the intestinal tract. This can cause diarrhea, constipation, vomiting and stomach pain.

Diagnosis

Diagnosing Behçet’s disease can be difficult. The disease is rare and its symptoms can be similar to those of other diseases.

Certain rare characteristics of this disease can be helpful in diagnosis, such as recurrent, simultaneous and multiple oral and genital ulcers. In addition, inflammation of the lungs’ arteries in Behçet’s disease may cause an aneurysm. This rarely occurs in any other condition.

Some doctors test for Behçet’s disease by pricking the skin with a needle. This is called a pathergy test. After one or two days, people with Behçet’s can develop a lump or nodule where the needle broke the skin.

A skin biopsy can also support the diagnosis. In a biopsy, a small piece of skin is removed to be examined in a laboratory.

No tests can confirm or rule out Behçet’s disease with great certainty. To diagnose the disease, doctors look for the key symptoms. These include recurring mouth sores, genital sores, skin rashes and eye inflammation.

Expected Duration

Behçet’s disease can be a chronic (long-lasting) condition. 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

Research regarding treatment of a condition as rare as Behçet’s disease is difficult to perform. Large, definitive studies have not been performed. Therefore, it is uncertain which treatment options are best. In general, treatment focuses on relieving the symptoms and depends on which parts of the body the disease affects.

• Rashes and sores in the mouth and genital area:
  • For mild disease — Corticosteroids (ointments, creams, rinses) applied to the skin (topical)
  • For more significant disease or if topical treatment is not successful — colchicine, dapsone (DDS) or thalidomide (Thalomid)
  • For more severe disease — Immune suppressants, including a pill form of corticosteroid, azathioprine (Imuran) or methotrexate (Methotrexate LPF, Rheumatrex)
  • For mouth pain — Novocaine-type agents. A mixture of Kaopectate, diphenhydramine (Benadryl) and lidocaine as a mouth rinse can provide 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, and immunosuppressants (including cyclosporine drops or pills, cyclophosphamide, and azathioprine)

Some studies suggest that interferon (Betaseron and others), adalimumab (Humira), etanercept (Enbrel), infliximab (Remicade) or rituximab (Rituxan) 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.

Vascular surgery may be necessary for aneurysms. This is particularly true if they are expanding and at risk for rupture. Blood thinners are usually recommended if blood clots complicate this disease.

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 can appear.

Additional Info

The American Behçet’s Disease Association
PO Box 869
Smithtown, NY 11787-0869
Phone: 1-800-723-4238
http://www.behcets.com/

American College of Rheumatology
1800 Century Place, Suite 250
Atlanta, GA 30345
Phone: 404-633-3777
Fax: 404-633-1870
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
Toll-Free: 1-800-283-7800
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: 1-877-226-4267
Fax: 301-718-6366
TTY: 301-565-2966
http://www.niams.nih.gov/