This page provides answers to questions about Behcets Disease (pronounced "BAY-sets") as well as the canker sores, mouth ulcers, and genital sores which frequently accompany it. You'll learn what the disease is and how it may develop. You'll also learn how it's diagnosed and treated. Following the article, you'll find a list of key words to help you understand the terms used on this page. If you have further questions after reading this article, you may wish to discuss them with your doctor.
The disease was first described in 1937 by Dr. Hulusi Behcet, a dermatologist in Turkey. Behcets disease is now recognized as 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.
The disease is common in the Middle East, Asia, and Japan; it is rare in the United States. In Middle Eastern and Asian countries, the disease affects more men than women. In the United States, the opposite is true. The 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 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, and pain. Doctors think 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.
The disease is not contagious; it is not spread from one person to another. Researchers think that two factors are important for a person to get it. First, it is believed that abnormalities of the immune system make some people susceptible to the disease. Scientists think that this susceptibility may be inherited; that is, it may be due to one or more specific genes. Second, something in the environment, possibly a bacterium or virus, might trigger or activate the disease in susceptible people.
This 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 Behcets disease. Symptoms can last for a long time or may come and go in a few weeks. Typically, symptoms appear, disappear, and then reappear, especially the canker sores, mouth ulcers, and genital sores. 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 Behcets disease and monitor its course, patients may want to keep a record of which symptoms occur and when. Because many conditions mimic Behcets disease, physicians must observe the lesions (injuries) caused by the disorder in order to make an accurate diagnosis.
The five most common symptoms of Behcets disease are canker sores or mouth ulcers, genital sores, other skin lesions, inflammation of parts of the eye, and arthritis.
In addition to canker sores, mouth ulcers, genital sores, other skin lesions, eye inflammation, and arthritis, Behcets disease may also cause blood clots, and inflammation in the central nervous system and digestive organs.
Blood Clots
About 16 percent of patients with this disease have blood clots resulting from inflammation in the veins (thrombophlebitis), usually in the legs. Symptoms include pain and tenderness in the affected area. The area may also be swollen and warm. Because thrombophlebitis can have severe complications, people should report symptoms to their doctor immediately. A few patients may experience artery problems such as aneurysms (balloon-like swelling of the artery wall).
Central Nervous System
Behcets disease affects the central nervous system in about 23 percent of all patients with the disease in the United States. The central nervous system includes the brain and spinal cord. Its function is to process heand coordinate thinking, behavior, sensation, and movement. The disease can cause inflammation of the brain and the thin membrane that covers and protects the brain and spinal cord. This condition is called meningoencephalitis. People with meningoencephalitis may have fever, headache, stiff neck, and difficulty coordinating movement, and should report any of these symptoms to their doctor immediately. If this condition is left untreated, a stroke (blockage or rupture of blood vessels in the brain) can result.
Digestive Tract
Rarely, the disease causes inflammation and ulceration (sores) throughout the digestive tract that are identical to the aphthous lesions in the mouth and genital area. This leads to abdominal pain, diarrhea, and/or bleeding. Because these symptoms are very similar to symptoms of other diseases of the digestive tract, such as ulcerative colitis and Crohn's disease, careful evaluation is essential to rule out these other diseases.
Diagnosing Behcets disease is very difficult because no specific test confirms it. Less than half of patients initially thought to have the 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 Behcets disease:
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 Behcets from other disorders. Because the disease affects different parts of the body, a patient probably will see several different doctors. It may be helpful to both the doctors and the patient for one doctor to manage the complete treatment plan. This doctor can coordinate the treatments and monitor any side effects from the various medications that the patient takes.
A rheumatologist (a doctor specializing in arthritis and other inflammatory disorders) often manages a patient's treatment and treats joint disease. The following specialists also treat other symptoms that affect the different body systems:
Although there is no cure, 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 Behcets disease.
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 Behcets symptoms because it suppresses the immune system and reduces inflammation throughout the body.
*Brand names included in this booklet are provided as examples only, and their inclusion does not mean that these products are endorsed by the National Institutes of Health or any other Government agency. Also, if a particular brand name is not mentioned, this does not mean or imply that the product is unsatisfactory.
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 Behcets disease keep their joints strong and flexible.
Most people with Behcets 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 Behcets disease.
Researchers are exploring possible genetic, bacterial, and viral causes of Behcets disease as well as improved drug treatment. For example, genetic studies show strong association of the gene HLA-B51 with the disease, but the exact role of this gene in the development of Behcets is uncertain. Researchers hope to identify genes that increase a person's risk for developing Behcets disease. Studies of these genes and how they work may provide new understanding of the disease and possibly new treatments.
Researchers are also investigating factors in the environment, such as bacteria or viruses, that may trigger Behcets disease. They are particularly interested in whether Streptococcus, the bacterium that causes strep throat, is associated with Behcets disease. Many people with Behcets disease have had several strep infections. In addition, researchers suspect that herpesvirus type 1, a virus that causes cold sores, may be associated with Behcets disease.
Finally, researchers are identifying other medicines to better treat Behcets disease. TNF inhibitors are a class of drugs that reduce joint inflammation by blocking the action of a substance called tumor necrosis factor. Although serious side effects have been reported for TNF inhibitors, they have shown some promise in treating Behcets disease. Examples of TNF inhibitors include etanercept and infliximab. Also, interferon alpha, a protein that helps fight infection, has shown promise in treating Behcets disease. Thalidomide, which is believed to be a TNF inhibitor, appears effective in treating severe mouth sores, but its use is experimental and side effects are a concern. Thalidomide is not used to treat women of childbearing age because it causes severe birth defects.
Anesthetic--Pain relief medicine that dulls feeling in the sore area. Doctors sometimes use an anesthetic to relieve pain caused by mouth and genital sores in people with Behcets disease.
Aneurysm--Swelling of an artery due to a weakness in its wall.
Antibody--A special protein produced by the body's immune system that recognizes and helps fight infectious agents and other foreign substances that invade the body.
Aphthosis--Ulcers in the mouth or on the genitals. Oral or mouth aphthosis, also called aphthous stomatitis, is the most common symptom of Behcets disease.
Arthritis--Literally means joint inflammation. It is a general term for more than 100 of the rheumatic diseases. Arthritis causes joint swelling, pain, and stiffness. Some people with Behcets disease develop a form of arthritis that goes away after a few weeks or months and causes no long-term damage to the joints.
Autoimmune disease--A disease that results when the immune system mistakenly attacks the body's own tissues.
Blood vessels--Arteries, veins, and capillaries that carry blood through the body.
Central nervous system--This body system includes the brain and spinal cord. Its functions are to process information and coordinate thinking, behavior, sensation, and movement. In some people with Behcets disease, blood vessels in the central nervous system become inflamed, causing headaches, stiff neck, and clumsiness.
Corticosteroids--Strong anti-inflammatory hormones that are made naturally in the body or synthetically (man-made) for use as medicine. They also are called glucocorticoids. The most commonly prescribed medicine of this type is prednisone.
Crohn's disease--Inflammation of the small intestine or colon that causes diarrhea, cramps, and weight loss.
Digestive tract--The body system that breaks down food. The digestive tract includes the stomach, intestines, pancreas, gallbladder, and liver.
Flare--A period of time when disease symptoms reappear or become worse.
Immune system--A complex network of specialized cells and organs that work together to defend the body against attacks by "foreign" invaders such as bacteria and viruses. In some rheumatic conditions, it appears that the immune system does not function properly and may even work against the body.
Immunosuppressive drugs--Medicines that reduce the immune response and therefore may relieve some symptoms of Behcets disease.
Inflammation--A reaction of tissues to injury or disease, marked by four signs: swelling, redness, heat, and pain.
Meningitis--Inflammation of the membranes that cover the brain and spinal cord.
Meningoencephalitis--Inflammation of the membranes that cover and protect the brain and spinal cord. This condition sometimes occurs in patients with Behcets disease.
Pathergy (a state of heightened reactivity)--A test that detects pathergic skin sensitivity is sometimes used to help diagnose Behcets disease. The skin is pricked with a small needle and in some people, a bump appears after 1 or 2 days. This reaction is called positive pathergy.
Reactive arthritis--A form of arthritis that can develop after an intestinal or urinary tract infection. The disease causes pain and swelling around the joints and in the spine. People with the disease may also experience swelling of the eye and the reproductive and urinary tracts.
Streptococcus--A bacterium that causes infections such as strep throat. Doctors think that it also may trigger Behcets disease in some people.
Thrombophlebitis--Inflammation of a vein and formation of a blood clot in the vein.
Topical treatment--Medicine, such as a cream or rinse, that is put directly on the affected body part.
Ulcerative colitis--Inflammation of the colon. Symptoms include stomach pain and diarrhea.
Uveitis--Inflammation of the inner eye that includes the iris, the tissue that holds the lens of the eye, and a network of blood vessels surrounding the eyeball called the choroid plexus.
The NIAMS gratefully acknowledges the assistance of Kenneth T. Calamia, M.D., of the Mayo Clinic, Jacksonville, Florida; Joseph L. Jorizzo, M.D., Wake Forest University Medical Center, Winston-Salem, North Carolina; Barbara Mittleman, M.D., NIAMS, NIH; and Roy S. Rogers, III, M.D., Mayo Clinic, Rochester, Minnesota, in the preparation and review of this booklet.
The mission of the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), a part of the Department of Health and Human Services' National Institutes of Health (NIH), is to support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases, the training of basic and clinical scientists to carry out this research, and the dissemination of information on research progress in these diseases. The National Institute of Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse is a public service sponsored by the NIAMS that provides health information and information sources. Additional information can be found on the NIAMS Web site at www.niams.nih.gov.
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