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Behcet's disease

Overview

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.

Symptoms

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 — 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.

Causes

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.

Risk factors

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.

Complications

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.

Diagnosis

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.

Treatment

No cure exists for Behcet's disease. If you have a mild form of the condition, your doctor may offer medications to control temporary flares in pain and inflammation. You may not need to take medication between flares.

If your signs and symptoms are more severe, your doctor may advise 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

Your doctor works to control any signs and symptoms you experience during flares with medications such as:

  • Skin creams, gels and ointments. Topical corticosteroid medicines are applied directly to skin and genital sores in order to reduce inflammation and pain.
  • Mouth rinses. Special mouthwashes that contain corticosteroids and other agents 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

If topical medications don't help, your doctor may recommend a drug called colchicine (Colcrys). Arthritis symptoms also may improve with colchicine.

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, 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 weight gain, persistent heartburn, high blood pressure and bone thinning (osteoporosis).

  • Medications that suppress your immune system. By stopping your immune system from attacking healthy tissues, immunosuppressive drugs reduce the inflammation. Immunosuppressive drugs that may play a role in controlling Behcet's disease include azathioprine (Imuran, Azasan), cyclosporine (Gengraf, Neoral, Sandimmune) and cyclophosphamide (Cytoxan).

    Because 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.

  • Medications that alter your immune system's response. Interferon alfa-2b (Intron A) regulates the activity of your immune system to control inflammation. It may be used alone or with other drugs to 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.

    Medications that block a substance called tumor necrosis factor (TNF) are effective in treating some of the signs and symptoms of Behcet's, especially for people who have more-severe or resistant symptoms. Examples include infliximab (Remicade) and etanercept (Enbrel).

    Side effects may include headache, skin rash and an increased risk of upper respiratory infections.

Preparing for an appointment

You're likely to start by seeing your primary care doctor if you have signs and symptoms of Behcet's disease. He or she may refer you to a doctor who treats arthritis and other rheumatic illnesses (rheumatologist) to confirm a diagnosis of Behcet's disease.

If a rheumatologist wasn't involved in your diagnosis, you may be referred to a rheumatologist for the management of Behcet's. Depending on your signs and symptoms, you may also need to see an eye doctor (ophthalmologist) for eye problems, a gynecologist or a urologist for genital sores, a dermatologist for skin problems, a gastroenterologist for digestive difficulties, or a neurologist for symptoms that involve the brain or central nervous system.

Because appointments can be brief and there's often a lot of ground to cover, it's a good idea to be prepared. Here's some information to help you get ready for your appointment.

What you can do

  • List any symptoms you're experiencing, including any that seem unrelated to the reason for which you scheduled the appointment.
  • List key personal information, including major stresses and recent life changes.
  • List all medications, vitamins and supplements that you're taking, including doses.
  • Ask a family member or friend to come with you. In addition to offering support, he or she can write down information from your doctor or other clinic staff during the appointment.
  • List questions to ask your doctor. Preparing a list of questions can help you make the most of your time together.

For Behcet's, some basic questions to ask your doctor include:

  • What's the most likely cause of my symptoms?
  • What are other possible causes for my symptoms?
  • What tests do I need? Do they require any special preparation?
  • Is my condition temporary or long lasting?
  • What are my treatment options, and which do you recommend?
  • I have another medical condition. How can I best manage these conditions together?
  • Do you have any brochures or other printed material that I can take with me? What websites do you recommend?

What to expect from your doctor

Examples of questions your doctor may ask, include:

  • What types of signs and symptoms have you been having and when did they start?
  • Do you have your symptoms all the time, or do they come and go?
  • How severe are your symptoms?
  • When did you begin experiencing symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, seems to worsen your symptoms?
  • Does anyone in your family have a similar illness?

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.

Last updated: July 29th, 2017

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