Behcet's Syndrome

Behçet's disease is a chronic condition that causes canker sores or ulcers in the mouth and on the genitals, and inflammation in parts of the eye. In some people, the disease also results in arthritis (swollen, painful, stiff joints), skin problems, and inflammation of the digestive tract, brain, and spinal cord.

Behçet's disease relatively rare in the United States. It is more common In Middle Eastern and Asian countries and is more likely to affect men than women. Behçet's disease tends to develop in people in their 20's or 30's, but people of all ages can develop this disease.

The exact cause of Behçet's disease is unknown. Most symptoms of the disease are caused by inflammation of the blood vessels. Inflammation is a characteristic reaction of the body to injury or disease and is marked by four signs:

  • swelling
  • redness
  • heat
  • pain

It is thought that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful "foreign" substances, such as germs, that enter the body. In an autoimmune reaction, the immune system mistakenly attacks and harms the body's own tissues.

Behçet's disease is not contagious.

What are the symptoms of Behçet's disease?

Behçet's disease affects each person differently. Some people have only mild symptoms, such as canker sores or ulcers in the mouth or on the genitals. Others have more severe signs, such as meningitis, which is an inflammation of the membranes that cover the brain and spinal cord. Meningitis can cause fever, a stiff neck, and headaches.

More severe symptoms usually appear months or years after a person notices the first signs of Behçet's disease. Symptoms can last for a long time or may come and go in a few weeks. Typically, symptoms appear, disappear, and then reappear. The times when a person is having symptoms are called flares. Different symptoms may occur with each flare; the problems of the disease often do not occur together. To help the doctor diagnose Behçet's disease and monitor its course, patients may want to keep a record of which symptoms occur and when. Because many conditions mimic Behçet's disease, physicians must observe the lesions (injuries) caused by the disorder in order to make an accurate diagnosis.

The five most common symptoms of Behçet's disease are mouth sores, genital sores, other skin lesions, inflammation of parts of the eye, and arthritis.

  • Mouth sores affect almost all patients with Behçet's disease. Individual sores or ulcers are usually identical to canker sores, which are common in many people. They are often the first symptom that a person notices and may occur long before any other symptoms appear. The sores usually have a red border and several may appear at the same time. They may be painful and can make eating difficult. Mouth sores go away in 10 to 14 days but often come back. Small sores usually heal without scarring, but larger sores may scar.
  • Genital sores affect more than half of all people with Behçet's disease and most commonly appear on the scrotum in men and vulva in women. The sores look similar to the mouth sores and may be painful. After several outbreaks, they may cause scarring.
  • Skin problems are a common symptom of Behçet's disease. Skin sores often look red or resemble pus-filled bumps or a bruise. The sores are red and raised, and typically appear on the legs and on the upper torso. In some people, sores or lesions may appear when the skin is scratched or pricked. When doctors suspect that a person has Behçet's disease, they may perform a pathergy test, in which they prick the skin with a small needle; 1 to 2 days after the test, people with Behçet's disease may develop a red bump where the doctor pricked the skin. However, only half of the Behçet's patients in Middle Eastern countries and Japan have this reaction. It is less commonly observed in patients from the United States, but if this reaction occurs, then Behçet's disease is likely.
  • Uveitis involves inflammation of the middle or back part of the eye (the uvea) including the iris, and occurs in more than half of all people with Behçet's disease. This symptom is more common among men than women and typically begins within 2 years of the first symptoms. Eye inflammation can cause blurred vision; rarely, it causes pain and redness. Because partial loss of vision or blindness can result if the eye frequently becomes inflamed, patients should report these symptoms to their doctor immediately.
  • Arthritis, which is inflammation of the joints, occurs in more than half of all patients with Behçet's disease. Arthritis causes pain, swelling, and stiffness in the joints, especially in the knees, ankles, wrists, and elbows. Arthritis that results from Behçet's disease usually lasts a few weeks and does not cause permanent damage to the joints.

In addition to mouth and genital sores, other skin lesions, eye inflammation, and arthritis, Behçet's disease may also cause blood clots, and inflammation in the central nervous system and digestive organs.

How is Behçet's disease diagnosed?

Diagnosing Behçet's disease is very difficult because no specific test confirms it. Less than half of patients initially thought to have Behçet's disease actually have it. When a patient reports symptoms, the doctor must examine the patient and rule out other conditions with similar symptoms. Because it may take several months or even years for all the common symptoms to appear, the diagnosis may not be made for a long time. A patient may even visit several different kinds of doctors before the diagnosis is made.

These symptoms are key to a diagnosis of Behçet's disease:

  • Mouth sores at least three times in 12 months
  • Any two of the following symptoms: recurring genital sores, eye inflammation with loss of vision, characteristic skin lesions, or positive pathergy (skin prick test)

Besides finding these signs, the doctor must rule out other conditions with similar symptoms, such as Crohn's disease and reactive arthritis. The doctor also may recommend that the patient see an eye specialist to identify possible complications related to eye inflammation. A dermatologist may perform a biopsy of mouth, genital, or skin lesions to help distinguish Behçet's from other disorders.

How is Behçet's disease treated?

Although there is no cure for Behçet's disease, people usually can control symptoms with proper medication, rest, exercise, and a healthy lifestyle. The goal of treatment is to reduce discomfort and prevent serious complications such as disability from arthritis or blindness. The type of medicine and the length of treatment depend on the person's symptoms and their severity.

It is likely that a combination of treatments will be needed to relieve specific symptoms. Patients should tell each of their doctors about all of the medicines they are taking so that the doctors can coordinate treatment.

Topical medicine

Topical medicine is applied directly on the sores to relieve pain and discomfort. For example, doctors prescribe rinses, gels, or ointments. Creams are used to treat skin and genital sores. The medicine usually contains corticosteroids (which reduce inflammation), other anti-inflammatory drugs, or an anesthetic, which relieves pain.

Oral medicine

Doctors also prescribe medicines taken by mouth to reduce inflammation throughout the body, suppress the overactive immune system, and relieve symptoms. Doctors may prescribe one or more of the medicines described below to treat the various symptoms of Behçet's disease.

  • Corticosteroids--Prednisone is a corticosteroid prescribed to reduce pain and inflammation throughout the body for people with severe joint pain, skin sores, eye disease, or central nervous system symptoms. Patients must carefully follow the doctor's instructions about when to take prednisone and how much to take. It also is important not to stop taking the medicine suddenly, because the medicine alters the body's production of the natural corticosteroid hormones. Long-term use of prednisone can have side effects such as osteoporosis (a disease that leads to bone fragility), weight gain, delayed wound healing, persistent heartburn, and elevated blood pressure. However, these side effects are rare when prednisone is taken at low doses for a short time. It is important that patients see their doctor regularly to monitor possible side effects. Corticosteroids are useful in early stages of disease and for acute severe flares. They are of limited use for long-term management of central nervous system and serious eye complications.
  • Immunosuppressive drugs--These medicines (in addition to corticosteriods) help control an overactive immune system, which occurs in Behçet's disease, and reduce inflammation throughout the body, and can lessen the number of disease flares. Doctors may use immunosuppressive drugs when a person has eye disease or central nervous system involvement. These medicines are very strong and can have serious side effects. Patients must see their doctor regularly for blood tests to detect and monitor side effects.

Doctors may use one or more of the following immunosuppressive drugs depending on the person's specific symptoms.

  • Azathioprine--Most commonly prescribed for people with organ transplants because it suppresses the immune system, azathioprine is now used for people with Behçet's disease to treat uveitis and other uncontrolled disease manifestations. This medicine can upset the stomach and may reduce production of new blood cells by the bone marrow.
  • Chlorambucil or cyclophosphamide--Doctors may use these drugs to treat uveitis and meningoencephalitis. People taking either agent must see their doctor frequently because either can have serious side effects, such as permanent sterility and cancers of the blood. Patients have regular blood tests to monitor blood counts of white cells and platelets.
  • Cyclosporine--Like azathioprine, doctors prescribe this medicine for people with organ transplants. When used by patients with Behçet's disease, cyclosporine reduces uveitis and uncontrolled disease in other organs. To reduce the risk of side effects, such as kidney and liver disease, the doctor can adjust the dose. Patients must tell their doctor if they take any other medicines, because some medicines affect the way the body uses cyclosporine.
  • Colchicine--Commonly used to treat gout, which is a form of arthritis, colchicine reduces inflammation throughout the body. The medicine sometimes is used to treat arthritis, mucous membrane, and skin symptoms in patients with Behçet's disease. A research study in Turkey suggested that the medication works best for males with the disorder. Common side effects of colchicine include nausea, vomiting, and diarrhea. The doctor can decrease the dose to relieve these side effects.
  • Combination treatment--Cyclosporine is sometimes used with azathioprine when one alone fails. Prednisone along with an immunosuppressive drug is a common combination.

If these medicines do not reduce the symptoms, doctors may use other drugs such as methotrexate. Methotrexate (Rheumatrex,Trexall), which is also used to treat various kinds of cancer as well as rheumatoid arthritis, can relieve Behçet's symptoms because it suppresses the immune system and reduces inflammation throughout the body.

Rest and exercise

Although rest is important during flares, doctors usually recommend moderate exercise, such as swimming or walking, when the symptoms have improved or disappeared. Exercise can help people with Behçet's disease keep their joints strong and flexible.

What is the prognosis for a person with Behçet's disease?

Most people with Behçet's disease can lead productive lives and control symptoms with proper medicine, rest, and exercise. Doctors can use many medicines to relieve pain, treat symptoms, and prevent complications. When treatment is effective, flares usually become less frequent. Many patients eventually enter a period of remission (a disappearance of symptoms). In some people, treatment does not relieve symptoms, and gradually more serious symptoms such as eye disease may occur. Serious symptoms may appear months or years after the first signs of Behçet's disease.

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© 2010 Vivacare. Last updated May 4, 2011.

Reference: National Institutes of Arthritis and Musculoskeletal and Skin Diseases.

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Additional Resources

Overview
Bechet's Disease Overview (link to American Behcet's Disease Association)
Behcet's Syndrome Handout (link to Intellihealth)
Conditions
Medications
En Espanol
Sindrome de Behcet (link to NIAMS)
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