HEART & BLOOD Blood/Lymphatic System • Hemophilia • Amyloidosis • Giant cell arteritis • Vasculitis • Thrombocytopenia (low platelet count) • Behcet's disease • Takayasu's arteritis • Wegener's granulomatosis • Idiopathic thrombocytopenic purpura • Churg-Strauss syndrome

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Behcet's (beh-CHETS) disease, also called Behcet's syndrome, 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. Behcet's 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 Behcet's 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.

Signs and 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:

• Mouth. 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. Skin lesions may occur in people with Behcet's 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.
• Genitals. 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.
• Eyes. Behcet's disease may cause inflammation in the eye — a condition called uveitis (u-ve-I-tis). 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.
• Joints. 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.
• Vascular system. 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.
• Digestive system. Behcet's disease may cause a variety of signs and symptoms that affect the digestive system, including abdominal pain, diarrhea or bleeding.
• Brain. 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 Behcet's 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 Behcet's 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.

Make an appointment with your doctor if you notice any unusual signs and symptoms that might indicate Behcet's disease. If you've been diagnosed with Behcet's disease, see your doctor if you notice any new signs and symptoms.

No tests can determine definitively whether or not you have Behcet's disease. Instead, your doctor relies primarily on your signs and symptoms to diagnose Behcet's disease. Your doctor may conduct blood tests or other laboratory tests to rule out other diseases and conditions.

Criteria have been established for the diagnosis of Behcet's disease, but these aren't always essential for the diagnosis of the disease. Your doctor may use other factors for your diagnosis. The criteria require mouth sores that recur three times in one year. In addition, you must meet two of the following criteria:

• Genital sores. Sores that recur may indicate Behcet's disease.
• Eye problems. An ophthalmologist can identify signs of inflammation in your eyes.
• Skin sores. A variety of rashes or acne-like sores may be caused by Behcet's disease.
• Positive pathergy test. In a pathergy test, your doctor inserts a sterile needle into your skin and then examines the area two days later. If the pathergy test is positive, a small red bump forms under your skin where the needle was inserted. This indicates your immune system is overreacting to the minor injury.

Behcet's disease typically comes and goes in unpredictable cycles. There's some indication that the disease becomes less severe after about 20 years. Though treatment can't cure Behcet's disease, it often can control signs and symptoms and reduce the risk of complications. For instance, untreated uveitis can lead to blindness. People with signs and symptoms of Behcet's disease in their eyes should be carefully monitored by an eye doctor. Other complications of Behcet's disease depend on the specific set of signs and symptoms you're experiencing.

No cure for Behcet's disease exists. If your signs and symptoms of Behcet's disease are mild, your doctor may offer medications to control temporary flares in pain and inflammation. You may not need to take medication between flares. But if your signs and symptoms are more severe, your doctor may advise systemic medications to control the signs and symptoms of Behcet's disease throughout your body, in addition to medications for the temporary flares.

Treatments for individual signs and symptoms of Behcet's disease
Behcet's disease may come and go on its own in fits of flares and remissions. Your doctor works to control any signs and symptoms you experience during flares with medications, such as:

• Skin creams, gels and ointments. Topical medicines are applied directly to skin and genital sores in order to reduce inflammation and pain. These types of medications usually contain a corticosteroid drug that reduces inflammation or an anesthetic to relieve pain.
• Mouth rinses. Special mouthwashes that contain corticosteroids to reduce the pain of mouth sores may ease your discomfort.
• Eyedrops. Eyedrops containing corticosteroids or other anti-inflammatory medicines can relieve pain and redness in your eyes if inflammation is mild.

Systemic treatments for Behcet's disease
Severe cases of Behcet's disease require treatments to control damage from the disease between flares. If you have moderate to severe Behcet's disease, your doctor may prescribe:

• Corticosteroids to control inflammation. Corticosteroids, such as prednisone, in combination with other medications may reduce the inflammation caused by Behcet's disease. The signs and symptoms of Behcet's disease tend to recur when corticosteroids are used alone, so doctors often prescribe them with another medication to suppress the activity of your immune system (immunosuppressives). Side effects of corticosteroids include stomach ulcers, high blood pressure and bone thinning (osteoporosis).
• Medications that suppress your immune system. Immunosuppressive drugs suppress your immune system, which overreacts in Behcet's disease. By stopping your immune system from attacking normal, healthy tissues in your body, immunosuppressive drugs reduce the inflammation that your immune system causes. Immunosuppressive drugs that may play a role in controlling Behcet's disease include azathioprine (Imuran, Azasan), cyclosporine (Sandimmune) and cyclophosphamide (Cytoxan). Since these medications suppress the actions of your immune system, they may increase your risk of infection. Other possible side effects include liver and kidney problems, low blood counts and high blood pressure.
• Medication that regulates your immune system. Interferon alfa (Roferon-A, Intron A) regulates the activity of your immune system to control inflammation. It may help control skin sores, joint pain and eye inflammation in people with Behcet's disease. Side effects include flu-like signs and symptoms, such as muscle pain and fatigue. Interferon alfa may be combined with other medications.

Other drugs that have been used to treat Behcet's disease include colchicine and methotrexate.

Take care of yourself to better cope with the ups and downs of Behcet's disease. Your approach to caring for yourself will depend on what signs and symptoms you're feeling on a particular day. In general, try to:

• Rest during flares. When signs and symptoms such as mouth sores and joint pain appear, take time for yourself. Be flexible and adjust your schedule when possible so that you can rest when you need it.
• Be active when you have the energy. Moderate exercise, such as walking or swimming, can make you feel better between Behcet's disease flares. Exercise strengthens your body and can improve your mood.