Disease: Behcet's disease
Behcet's (beh-CHETS) disease, also called Behcet's syndrome, is a rare disorder that causes blood vessel inflammation throughout your body.
The disease can lead to numerous signs and symptoms that may seem unrelated at first. They may include mouth sores, eye inflammation, skin rashes and lesions, and genital sores. The effects of Behcet's disease vary from person to person and may clear up on their own.
Treatment involves medications to reduce the signs and symptoms of Behcet's disease and to prevent serious complications, such as blindness.
Behcet's disease symptoms vary from person to person. It may disappear and recur on its own. Symptoms may become less severe over time. Your signs and symptoms depend on which parts of your body are affected.
Areas commonly affected by Behcet's disease include:
- Mouth. Painful mouth sores that look similar to canker sores are the most common sign of Behcet's disease. They begin as raised, round lesions in the mouth that quickly turn into painful ulcers. The sores usually heal in one to three weeks, though they do recur.
- Skin. 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, especially on the lower legs.
- Genitals. People with Behcet's disease may develop sores on their genitals. The red, open sores commonly occur on the scrotum or the vulva. The sores are usually painful and may leave scars.
- Eyes. Behcet's disease may cause inflammation in the eye â a condition called uveitis (u-vee-I-tis). Uveitis causes redness, pain and blurred vision in one or both eyes. In people with Behcet's disease, the condition and may come and go.
- Joints. Joint swelling and pain often affect the knees in people with Behcet's disease. The ankles, elbows or wrists also may be involved. Signs and symptoms may last one to three weeks and go away on their own.
- Vascular system. Inflammation in blood vessels (veins and arteries) may occur in Behcet's disease, causing redness, pain, and swelling in the arms or legs when a blood clot results. Inflammation in the large arteries can lead to complications, such as aneurysms and narrowing or blockage of the vessel.
- Digestive system. Behcet's disease may cause a variety of signs and symptoms that affect the digestive system, including abdominal pain, diarrhea and bleeding.
- Brain. Behcet's disease may cause inflammation in the brain and nervous system that leads to headache, fever, disorientation, poor balance or stroke.
When to see a doctor
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 one knows exactly what causes Behcet's disease. But it may be an autoimmune disorder, which means the body's immune system mistakenly attacks some of its own healthy cells. It's likely that genetic and environmental factors also play a role. Several genes have been found to be associated with the disease. Some researchers believe a virus or bacterium may trigger Behcet's disease in people who have certain genes that make them susceptible to Behcet's.
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. He or she may conduct blood tests or other laboratory tests to rule out other 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 classification criteria require:
- Mouth sores. Because nearly everyone with Behcet's will have mouth sores at some point, this sign is generally necessary for a diagnosis. The diagnostic criteria require mouth sores that have recurred at least three times in 12 months.
In addition, to receive a diagnosis of Behcet's disease you must have at least two additional signs, such as:
- 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 one to 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 a minor injury.
Complications of Behcet's disease depend on your signs and symptoms. For instance, untreated uveitis can lead to decreased vision or even blindness. People with eye signs and symptoms of Behcet's disease need to visit an eye doctor (ophthalmologist) regularly because treatment can help prevent this complication.
Coping and support
The unpredictability of Behcet's disease can make it particularly frustrating. Taking good care of yourself may help you better cope. 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 appear, take time for yourself. Be flexible and adjust your schedule when possible so that you can rest when you need it. It's also a good idea to keep stress to a minimum, because stress can aggravate your symptoms.
- 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, helps keep your joints flexible and can improve your mood.
- Connect with others. Because Behcet's is a rare disorder, it may be difficult to find others with the disease nearby. Ask your doctor about support groups in your area. If it's not possible to connect with someone close, the American Behcet's Disease Association offers message boards and chat rooms where you can connect with other people who have Behcet's.
Factors that may increase your risk of Behcet's include:
- Age. Behcet's disease commonly affects men and women in their 20s and 30s, though children and older adults also can develop the condition.
- Where you live. People from countries in the Middle East and Far East, including Turkey, Iran, Japan and China, are more likely to develop Behcet's.
- Sex. While Behcet's disease occurs in both men and women, the disease is usually more severe in men.
- Genes. Having certain genes is associated with a higher risk of developing Behcet's.
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