Behcet’s syndrome was first described in 1937 by Dr. Helusi Behcet, a professor of dermatology in Istanbul. Behcet’s syndrome most commonly causes sores or ulcers in the mouth and on the genitals, and inflammation in parts of the eye. In some people however, it may also results in arthritis (swollen, painful, stiff joints) and inflammation of the digestive tract, brain, or spinal cord.
Behcet’s syndrome is common in the Middle East, Asia, and Japan, but rare in the United States. It affects men more than women. Although it can affect people in at all ages, typically it is diagnosed in people in their twenties or thirties.
Many clinicians and researchers classify it as a vasculitis, causing inflammation of the blood vessels, particularly veins. The exact cause is unknown, nor do we know what triggers the condition. Two factors are thought to be important. The first being inheritance of a specific genetic marker (HLA-B51) and the other, a possible environmental trigger (ie. bacterial or viral infection.) Behcet’s syndrome is not contagious and does not spread from one person to another.
Behcet’s syndrome varies among different individuals. From mild skin, oral or genital sores to more severe organ involvement. More severe symptoms usually appear months or years after a person notices the first signs of Behcet’s syndrome.
The four most common symptoms of Behcet’s syndrome are mouth sores, genital sores, inflammation of parts of the eye, and arthritis.
- Mouth sores- Affect almost all patients with Behcet’s syndrome and are often the first symptom. They have a characteristic red border and several may appear at the same time. They are often painful and can make eating difficult. Mouth sores typically resolve within 10 to 14 days but often recur.
- Genital sores- Affect more that half of all people with Behcet’s syndrome, appearing on the scrotum in men and vulva in women. They are similar in appearance to mouth sores and are often painful.
- Uveitis (yoo.vee.EYE.tis)- Inflammation of the middle part of the eye occurs in more than half of all people with Behcet’s syndrome. Symptoms include blurred vision and eye pain and redness. If untreated it may lead to partial vision loss or blindness.
- Arthritis- Inflammation of the joints occurs in more than half of all patients with Behcet’s syndrome. Arthritis causes pain, swelling, and stiffness in the joints, particularly the knees, ankles, wrists, and elbows. Arthritis from Behcet’s syndrome typically does not cause permanent damage to the joints.
- Vasculitis- Inflammation of blood vessels and can cause small strokes, severe skin ulcers mouth or genital ulcers, blood clots, or in severe cases rupture of large blood vessels in the lung, chest, or abdomen.
- Behcet’s syndrome may also cause
- Skin findings
- Disease of the brain and spinal cord
- Disease of the digestive tract
There are no confirmatory tests for diagnosing Behcet’s syndrome. The doctor must examine a patient with the appropriate clinical symptoms and rule out other similar conditions. There is often a lag between symptom onset and diagnosis as the symptoms are non-specific and often time take years to develop.
These symptoms are key to diagnosing Behcet’s syndrome:
- Mouth sores at least three times in 12 months AND
- Any two of the following symptoms:
- Recurring genital sores
- Eye inflammation with loss of vision
- Skin lesions, or positive pathergy (skin prick test)
Similar conditions to rule out: Crohn’s syndrome; Reactive arthritis
Treatment goals are to reduce inflammation and thereby reduce discomfort and prevent serious complications. There is no cure, however symptoms can be controlled through proper medication, rest, and exercise. The type of medicine and the duration of treatment depend on the person’s symptoms and their severity.
Topical medications are applied directly on the sores to relieve pain and discomfort. These include oral rinses or topical ointments or creams. They often contain corticosteroids, which reduce inflammation, or an anesthetic, which relieves pain.
Often required to relieve symptoms and suppress inflammation. One or more of the following medicines may be used treat the various symptoms of Behcet’s syndrome.
- Prednisone is a corticosteroid used to reduce pain and inflammation throughout the body in people with severe joint pain, skin sores, eye involvement, or central nervous system symptoms.
Depending on the person’s specific symptoms, doctors may use one or more of the following immunosuppressive drugs, typically for more severe disease:
- Colchicine- Commonly used to treat gout, colchicine reduces inflammation throughout the body. It is used to treat oral and genital sores as well as eye inflammation and skin symptoms.
- Azathioprine- Most commonly prescribed for organ transplantion, azathioprine is used to treat Behcet’s eye inflammation and central nervous system involvement.
- Cyclosporine- Like azathioprine, it is commonly used for organ transplantation. Cyclosporine reduces eye inflammation and central nervous system involvement.
- IT IS IMPORTANT TO DISCUSS THE APPROPRIATE WAY TO TAKE EACH OF THESE MEDICATIONS AND TO DISCUSS THEIR SHORT AND LONG TERM RISKS/SIDE EFFECTS WITH YOUR PRESCRIBING PHYSICIAN
If these medicines do not reduce symptoms, doctors may use other drugs such as cyclophosphamide and methotrexate.
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 resolved. Exercise and physical therapy can help people with Behcet’s syndrome keep their joints strong and flexible.
Most people with Behcet’s syndrome can lead normal lives and control their symptoms with proper medicine, rest, and exercise. Many patients eventually enter a period of remission. In some patients however, treatment does not alleviate symptoms, and gradually complications such as eye involvement may develop. Serious symptoms may appear months or even years after the first signs of Behcet’s syndrome.
* Adopted from MD Consult Patient Handouts and The National Institute of Arthritis and Musculoskeletal and Skin Syndromes site