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Behcets Disease, also called Behcet's Disease, is a rare disorder that causes chronic inflammation in blood vessels throughout the body. The inflammation of Behcet's disease leads to a variety of signs and symptoms that may seem unrelated.
Behcet's disease most commonly affects men and women in their 20s and 30s, though children and older adults can also develop the condition. Behcets disease is most common in countries in the Middle East and Asia, including Turkey, Iran, Japan and China. While Behcet's disease occurs in both men and women, the disease is usually more severe in men. Behcet's disease is also typically more severe when it occurs at an earlier age.
No cure exists for Behcet's disease. The signs and symptoms of Behcets disease — which may include mouth sores, skin rashes and lesions, and genital sores — vary from person to person and may come and go on their own. Treatment aims to reduce the signs and symptoms of Behcet's disease and to prevent serious complications, such as blindness.
Symptoms of Behcet's disease vary from person to person. The signs and symptoms that you may experience depend on what parts of your body are affected by the inflammation of Behcet's disease. Body areas most commonly affected by Behcet's disease include:
Painful mouth sores, identical to canker sores, are the most common sign of Behcet's disease. Sores begin as raised, round lesions on the lips, gums, cheeks and tongue. After a day or two, the sores become shallow ulcers and a white or yellow covering develops. The sores heal usually in seven to 10 days, though they frequently recur. Many mouth sores may occur at the same time. Sores may be more persistent in severe cases.
Skin lesions may occur in people with Behcets disease. Skin problems can vary. Some people may develop acne-like sores on their bodies. Others may develop red, raised and tender nodules on their skin. Skin problems may clear up on their own in 10 to 14 days, though they usually recur.
People with Behcet's disease may develop sores on their genitals. The sores most commonly occur on the penis or the vulva. Sores appear as round, red and ulcerated lesions. The genital sores are usually painful and may leave scars.
Behcet's disease may cause inflammation in the eye — a condition called uveitis. In people with Behcet's disease, uveitis causes redness, pain and blurred vision in one or both eyes and may come and go. Inflammation that occurs in the blood vessels of the retina is a serious complication of the disorder.
Joint swelling, redness and pain most commonly affect the knee in people with Behcet's disease. The ankle, elbow or wrist may also be involved. Signs and symptoms may last one to three weeks and go away on their own.
Inflammation in veins and large arteries may occur in Behcet's disease, causing redness, pain and swelling in the arms or legs. These signs and symptoms may come and go, and they may move from one limb to another. Inflammation in the large arteries can lead to complications such as aneurysms and blood clots.
Behcets disease may cause a variety of signs and symptoms that affect the digestive system, including abdominal pain, diarrhea or bleeding.
Behcet's disease may cause inflammation in the brain and nervous system that leads to headache, fever, disorientation, poor balance or stroke.
Behcet's disease may recur on its own. Signs and symptoms may disappear for one or two months and then return.
Doctor's don't know what causes Behcet's disease. Many believe Behcets disease is a form of autoimmune disorder in which the body's defense system — the immune system — turns on itself. Rather than attack foreign invaders, such as bacteria and viruses, the immune system attacks healthy cells in the body.
Behcet's disease is likely caused by a combination of genetic and environmental factors. Some researchers believe a virus or bacterium may trigger Behcets disease in people who have a certain genetic background. Other possible triggers that could set off the immune system could include chemicals or heavy metals.
The treatment of Behcet's syndrome depends on the severity and the location of its manifestations in an individual patient.
Steroid gels, pastes 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. 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 or oral steroids. Colchicine and oral and injectable cortisone are used for inflammation involving the joints, eyes, skin, and brain. Sulfasalazine 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 Remicade and 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 and cyclophosphamide, 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, 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.