Behcet's Syndrome

Medical Author: William C. Shiel Jr., MD, FACP, FACR

• What is Behcet's syndrome?
• What are symptoms of Behcet's syndrome?
• How is Behcet's syndrome diagnosed?
• What is the treatment of Behcet's syndrome?
• Behcet's Syndrome At A Glance

What is Behcet's syndrome?

Behcet's syndrome is classically characterized as a triad of symptoms that include recurring crops of mouth ulcers (aphthous ulcers), genital ulcers, and inflammation of a specialized area around the pupil of the eye (the uvea). The inflammation of the area of the eye that is around the pupil is called uveitis. Behcet's syndrome is also sometimes referred to as Behcet's disease.

The cause of Behcet's syndrome is not known. The disease is more frequent and severe in patients from the Eastern Mediterranean and Asia than those of European descent. Both inherited (genetic) and environmental factors, such as microbe infections, are suspected to be factors that contribute to the development of Behcet's syndrome. Behcet's syndrome is not felt to be contagious.

What are symptoms of Behcet's syndrome?

The symptoms of Behcet's syndrome depend on the area of the body affected. Behcet's syndrome can involve inflammation of many areas of the body. These areas include the arteries that supply blood to the body's tissues. Behcet's syndrome can also affect the veins that take the blood back to the lungs to replenish the oxygen content. Other areas of body that can be affected by the inflammation of Behcet's syndrome include the back of the eyes (retina), brain, joints, skin, and bowels.

The mouth and genital ulcers are generally painful and recur in crops (many shallow ulcers at the same time). They range in size from a few millimeters to 20 millimeters in diameter. The mouth ulcers occur on the gums, tongue, and inner lining of the mouth. The genital ulcers occur on the scrotum and penis of males and vulva of women and can leave scars.

Inflammation of the eye, which can involve the front of the eye (uvea) causing uveitis, or the back of the eye (retina) causing retinitis, can lead to blindness. Symptoms of eye inflammation include pain, blurred vision, tearing, redness, and pain when looking at bright lights. It is very important for patients to have this sensitive area monitored by an eye specialist (ophthalmologist).

If the arteries become inflamed (arteritis) in patients with Behcet's syndrome, it can lead to death of the tissues whose oxygen supply depends on these vessels. This could cause a stroke if affecting the brain, belly pain if affecting the bowel, etc. When veins become inflamed (phlebitis), the inflammation can involve large veins that develop blood clots which can loosen to cause pulmonary embolism.

Symptoms of inflammation of the brain or tissue that covers the brain (meninges) include headaches, neck stiffness, and is often associated with fever. Inflammation of the brain (encephalitis) and/or the meninges (meningitis) can cause damage to nervous tissue and lead to weakness or impaired function of portions of the body. This can result in confusion and coma. Typically these features occur later in the disease course, years after the diagnosis.

Joint inflammation (arthritis) can lead to swelling, stiffness, warmth, pain, and tenderness of joints in patients with Behcet's syndrome. This occurs in about half of patients with Behcet's syndrome at sometime during their lives. Knees, wrists, ankles, and elbows are the most common joints affected.

The skin of patients with Behcet's syndrome can develop areas of inflammation which spontaneously appear as raised, tender, reddish nodules (erythema nodosum), typically on the front of the legs. Some patients with Behcet's syndrome develop a peculiar red or blistery skin reaction in places where they have been pierced by blood-drawing needles (see pathergy test in diagnosis section below). Recent research has found that acne occurs more frequently in patients with Behcet's syndrome that also have arthritis as a manifestation.

Ulcerations can occur at any location in the stomach, large or small bowel in patients with Behcet's disease.

Next: How is Behcet's syndrome diagnosed? »

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