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Medical Author:

William C. Shiel Jr., MD, FACP, FACR

William C. Shiel Jr., MD, FACP, FACR

Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

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In this Article

• What is Behcet's syndrome?
• What are symptoms of Behcet's syndrome?
• How is Behcet's syndrome diagnosed?
• What is the treatment of Behcet's syndrome?
• Behcet's Syndrome At A Glance
• Patient Comments: Behcet&39;s Syndrome - Symptoms
• Behcet's Syndrome Index

Behcet's syndrome is diagnosed based on the finding of recurrent mouth ulcerations combined with any two of the following: eye inflammation, genital ulcerations, or skin abnormalities mentioned above. A special skin test called a pathergy test can also suggest Behcet's syndrome. (The other criteria above are still required for ultimate diagnosis.) This test consists of pricking the skin of the forearm with a sterile needle. The test is called positive and suggests Behcet's syndrome when the puncture causes a sterile red nodule or pustule that is greater than two millimeters in diameter at 24 to 48 hours.

Tests such as skin biopsy, lumbar puncture, MRI scan of the brain, and bowel tests are considered based upon symptoms that are present.

The treatment of Behcet's syndrome depends on the severity and the location of its manifestations in an individual patient.

Steroid (cortisone) gels, pastes (such as Kenolog in Orabase) and creams can be helpful for the mouth and genital ulcers. Colchicine can also minimize recurrent ulcerations. Mouth and genital ulcers healed and were reported at a national meeting of the American College of Rheumatology as less frequent in nine or 12 patients who were treated with Trental (pentoxifylline). Trental also seemed to maintain the healed ulcers for up to the 29 months of the study. The effectiveness of Trental, the researchers said, seemed to be enhanced by the combination with colchicine in some patients.

Joint inflammation can require nonsteroidal anti-inflammatory drugs (such as ibuprofen and others) or oral steroids. Colchicine and oral and injectable cortisone are used for inflammation involving the joints, eyes, skin, and brain. Sulfasalazine (Azulfidine) has been effective in some patients for arthritis.

Bowel disease is treated with oral steroids and sulfasalazine.

Diligent treatment of eye inflammation is essential. Patients with eye symptoms or a history of eye inflammation should be monitored by an ophthalmologist. Recent research has reported successful management of resistant eye inflammation with new biologic medications that block a protein that plays a major role in initiating inflammation, called TNF. These TNF-blocking medications, including infliximab (Remicade) and etanercept (Enbrel), can also be helpful for severe mouth ulcerations.

Severe disease of the arteries, eyes, and brain can be difficult to treat and require powerful medications that suppress the immune system called immunosuppressive agents. Immunosuppressive agents used for severe Behcet's syndrome include chlorambucil (Leukeran), azathioprine (Imuran), and cyclophosphamide (Cytoxan). Cyclosporine has been used for resistant disease.

Recent studies suggest that thalidomide may be of benefit for certain patients with Behcet's syndrome in treating and preventing ulcerations of the mouth and genitals. Side effects of thalidomide include promoting abnormal development of fetal growth, nerve injury (neuropathy), and hypersedation.

Recent research further suggests that patients who have had heart-valve replacement because of severe damage as a result of inflammation can benefit by immune suppression with azathioprine and prednisone after operation.

Trials are currently underway evaluating interferon alpha for the treatment of eye disease in patients with Behcet's syndrome.

• Behcet's syndrome is associated with inflammation of various areas of the body.
• Symptoms of Behcet's syndrome depend on the body areas affected.
• Recurrent mouth ulcers are characteristic of Behcet's syndrome.
• Treatment of Behcet's syndrome depends on the severity and the location of its manifestations.

References:

Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.

Kelley's Textbook of Rheumatology, W B Saunders Co, edited by Shaun Ruddy, et al., 2000.

American College of Rheumatology, Annual Scientific Meeting; November, 2006, 2007.