Section 5. Bone, Joint, and Muscle Disorders

Chapter 69. Vasculitic Disorders of Connective Tissue

Topics: Introduction | Polyarteritis Nodosa | Temporal Arteritis | Polymyalgia Rheumatica | Wegener's Granulomatosis | Behçet's Syndrome

Behçet's Syndrome

Behçet's syndrome is a chronic, relapsing inflammatory disorder that can produce recurring, painful mouth sores, skin blisters, genital sores, and swollen joints; the eyes, blood vessels, nervous system, and digestive tract may also become inflamed.

This syndrome affects men twice as often as women. It usually appears in people during their 20s, but sometimes it develops in childhood. Behçet's syndrome is uncommon in the United States. People from Mediterranean countries, Japan, Korea, and the area along the silk route through China are at highest risk. The cause of Behçet's syndrome is unknown, but viruses and autoimmune disorders may play a role.

Symptoms

Almost everyone with this syndrome has recurring, painful mouth sores, similar to canker sores. Sores may also appear on the penis, scrotum, and vulva and tend to be painful; those in the vagina may be painless.

Other symptoms appear days to years later. A recurring inflammation of part of the eye (relapsing iridocyclitis) produces eye pain, sensitivity to light, and hazy vision. Several other eye problems can occur; one of them, uveitis (see Section 20, Chapter 232), can cause blindness if untreated.

Skin blisters and pus-filled pimples develop in about 80% of people. A minor injury, even a puncture from a hypodermic needle, can cause the area to become swollen and inflamed. About half of the people develop a relatively mild, nonprogressive joint inflammation (arthritis) in the knees and other large joints. Vasculitis throughout the body can cause blood clot formation, aneurysms (bulges in weakened blood vessel walls), strokes, and kidney damage. When the digestive tract is affected, symptoms may range from mild discomfort to severe cramping and diarrhea.

The recurring symptoms of Behçet's syndrome can be very disruptive. The symptoms or the symptom-free periods (remissions) may last weeks, years, or decades. Occasionally, damage to the nervous system, digestive tract, or blood vessels is fatal.

Diagnosis and Treatment

The diagnosis is based on the physical examination because no laboratory tests can detect Behçet's syndrome. However, symptoms resemble those of many other diseases, including Reiter's syndrome (reactive arthritis), Stevens-Johnson syndrome, systemic lupus erythematosus, Crohn's disease, herpes infection, and ulcerative colitis.

Although there is no cure, specific symptoms can usually be relieved by treatment. For example, a corticosteroid applied externally (rather than by mouth) can help heal inflamed eyes and skin sores. People who develop a severe inflammation of the eyes or the nervous system may need to be treated with prednisone or another corticosteroid. Cyclosporine, an immunosuppressive drug, may be given when the eye problems are severe or when prednisone does not adequately control symptoms. Colchicine given by mouth in low doses may help prevent oral and genital ulcers. Thalidomide is being investigated for use. Needle punctures should be avoided because the skin may become inflamed.