• Behcet’s Disease

    (Adamantiales-Behcet’s Syndrome)

    Definition

    Behcet’s disease is a rare, chronic disorder involving inflammation of blood vessels throughout the body. It is marked by recurrent oral and genital ulcers and eye inflammation.

    Causes

    The cause of Behcet’s disease is unknown. It is believed to be caused by an autoimmune reaction. This occurs when the body’s immune system attacks its own tissue by mistake. This autoimmune abnormality may be inherited. An environmental trigger, such as a specific virus or bacterium, may activate the disease in people susceptible to it.

    Risk Factors

    Although the exact cause of Behcet’s disease is unknown, some groups of people are more likely to develop the condition than others. Factors that may increase your risk of Behcet's include:
    • Location: the Middle East, Asia, and Japan
    • Sex:
      • In the US, men are more likely than women to develop this condition.
      • In the Middle East, Asia, and Japan, women are more likely than men to develop Behcet’s.
    • Age: 20s and 30s

    Symptoms

    Symptoms of Behcet’s disease can vary from mild to very severe. Symptoms tend to appear, heal, and then recur (referred to as a flare) frequently over months or years. The most common symptoms of the disease are:
      Oral sores
      • Affect almost all people with the disease
      • Usually are the first to appear
      • Usually last for 10 to 14 days
      • Can be painful
      • Can cause scarring
      Ulcerated Gums
      Periodontal Disease
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      Genital sores
      • In men: appear on the penis and/or scrotum
      • In women: appear on the vulva or within the vagina
      • Can be painful
      • Can cause scarring
      Uveitis—inflammation of the middle part of the eye, including the iris
      • Tends to cause eye redness, blurred vision, sensitivity to light, and watering eyes
      • If not treated, can cause partial vision loss or blindness
      Arthritis —inflammation of joints
      • Tends to be painful
      • Usually does not cause permanent joint damage
      Skin problems, such as sores
      • May appear as red, raised sores or bumps, or may be flat (flush with the skin)
      • Usually appear on the legs and upper torso
    Other symptoms of the disease may include:
    • Severe fatigue during a flare
    • Blood clots
      • Caused by thrombophlebitis (inflammation of veins), usually in the legs
      • Can cause severe complications if not treated quickly
      Aneurysms , or severely dilated blood vessels
      • May rupture and cause severe consequences
      Heart problems, such as:
      • Abnormal heart rhythms
      • Missed heartbeats
      • Early heartbeats
      • Inflammation of the heart muscle ( myocarditis )
      Central nervous system problems
      • For example, meningoencephalitis, which is inflammation of the brain and membrane that lines the brain
      • May result in seizures , confusion, strokes , memory problems, headaches
      Stroke from a Cerebral Aneurysm
      Cerebral Aneurysm
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      Digestive problems
      • Causes ulcers and inflammation of the digestive tract (rarely)

    Diagnosis

    Diagnosing Behcet’s disease is very difficult because:
    • Symptoms are similar to those of many other diseases.
    • Symptoms often appear very slowly, over months or years.
    • There is no specific test to confirm the disease.
    A doctor may suspect Behcet’s disease if oral sores appear at least three times within a year, and at least two of the following recurring symptoms appear:
    • Eye inflammation
    • Genital sores
    • Skin sores
    If Behcet’s disease is suspected, your doctor will do tests to rule out other diseases with similar symptoms. Also, you may have a pathergy skin test. For this test, your skin is pricked with a small needle. If you have Behcet’s disease, a bump will develop at the site of the skin prick. However, this test is not conclusive. Many people with the disease do not have a reaction to the skin prick.

    Treatment

    There is no cure for Behcet’s disease however spontaneous regression may occur. Treatment is aimed at limiting and preventing complications from symptoms. With proper treatment, the disease can usually be managed fairly well. Treatment includes medications, rest, and exercise.

    Medication

    Both topical and oral medications are used.
    Topical medications include creams, lotions, and mouth rinses that contain corticosteroids (to cut down on inflammation) and/or anesthetics (to decrease pain). These are applied to sores to reduce inflammation and pain.
    Oral medications, include:
    • Oral corticosteroids—to reduce inflammation and pain
    • Immunosuppressive drugs—to help control the overactive immune system. These may include:
      • Interferon
      • Azathioprine
      • Chlorambucil
      • Cyclosporine
      • Colchicine
      • Methotrexate
      • Dapsone
      • Infliximab
      • Thalidomide
    Many of the drugs used to treat Behcet’s disease can cause severe side effects. If you are taking these medications your doctor must closely monitor you.

    Rest and Exercise

    General guidelines include:
    • Rest when symptoms flare to help speed healing.
    • Engage in moderate exercise when symptoms recede to help keep joints flexible and strong.

    Prevention

    There are no guidelines for the prevention of Behcet's disease because the exact cause is unknown.

    RESOURCES

    American Behcet’s Disease Association http://www.behcets.com/

    National Institute of Arthritis and Musculoskeletal and Skin Diseases http://www.niams.nih.gov/

    CANADIAN RESOURCES

    The Arthritis Society http://www.arthritis.ca/

    Health Canada http://www.hc-sc.gc.ca/

    References

    Abu El Asrar, ABoooud EB, ALbibhi H, Al-Arfaj A. Long-term safety and efficacy of infliximab therapy in refractory uveitis due to Behcet's disease. Int Ophthalmol . Sep 23, 2006.

    Cakir O, Eren N, Ulka R, Nazaroghi H. Bilateral subclavian arterial aneurysm and ruptured abdominal aorta pseudoaneurysm in Behcet's disease. Ann Vasc Surg. 2002,16:516-520

    The Medical Letter. 1996;38:15.

    The Merck Manual of Diagnosis and Therapy . Simon & Schuster; 2001.

    National Organization of Rare Disorders, Inc. website. Available at: http://www.rarediseases.org/ .

    National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: http://www.niams.nih.gov .

    Okada AA. Behcet's disease: general concepts and recent advances. Curr Opin Ophthalmol . 2006;17:551-556.

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