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Behcet Syndrome Symptoms and Causes of Behcet Syndrome
Article Summary: Behcet's syndrome is a multisystem autoimmune disease that involves inflammation of blood vessels throughout the body. It is characterized by recurring, painful mouth sores, skin blisters, genital sores and swollen joints. This disease affects men twice as often as women. It usually appears in people during their 20's, but develops sometimes in childhood. The disease is found worldwide, but it is most common in the Eastern Mediterranean countries and in eastern Asia where it is a leading cause of blindness. Behcet's syndrome is a chronic disorder in which many body systems including the eyes,
Behcet's syndrome is a multisystem autoimmune disease that involves inflammation of blood vessels throughout the body. It is characterized by recurring, painful mouth sores, skin blisters, genital sores and swollen joints. This disease affects men twice as often as women. It usually appears in people during their 20's, but develops sometimes in childhood. The disease is found worldwide, but it is most common in the Eastern Mediterranean countries and in eastern Asia where it is a leading cause of blindness. Behcet's syndrome is a chronic disorder in which many body systems including the eyes, the joints, the blood vessels, the central nervous system, the digestive system and the genitals become swollen and tender (inflamed).
Symptoms of Behcet Syndrome
Most of the symptoms are painful but not life-threatening. They come and go in a series of attacks (flare-ups) throughout life. The disease does not burn itself out, but sometimes it can level off and the flare-ups become less aggressive and happen less frequently. Most people with Behçets disease have a normal lifespan and can hope to lead close-to-normal daily lives.
Women and girls may get ulcers on the vulva, in the vagina or on the cervix. In men and boys ulcers affect the scrotum and/or the penis. Apart from ulcers, some men and boys experience pain or swelling in one or both testicles. Ulcers and boils may occur in both sexes around the anus and in the groin. Neither oral nor genital ulcers in Behçet's syndrome are caused by the herpes virus.
Swelling and pain in a blood vessel anywhere in the body. This can lead to aneurysms (weak places in the vessel that thin and bulge out) or blood clots. The tiny blood vessels of the kidneys may be affected without symptoms being apparent (asymptomatic focal glomerulonephritis).
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.
Causes of Behcet Syndrome
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.
No one knows why the immune system starts to behave this way in Behçet disease. It is not because of any known infections, it is not definitively hereditary but first degree relatives are affected in more than expected proportion of patients.
Patients who have a parent with Behçet syndrome have disease onset at a younger age (genetic anticipation). In addition, pediatric patients are more likely to have a family history of Behçet syndrome, compared to patients with disease onset as an adult.
The syndrome generally begins when a person is in his or her thirties. Men are twice as likely as women to develop it. People whose families once lived along the ancient silk route in Mediterranean and some Asian countries, especially Japan, have a greater risk of developing Behçet's syndrome.
The mouth and genital ulcers tend to occur in multiples and can be quite painful. In the mouth, these ulcers are generally found on the tongue, gums, and the inside of the lips or jaws. In the genital area, the ulcers usually occur on the penis and scrotum in males and on the vulva of women. The eye inflammation can lead to blindness.
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Peter Hutch
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