Behcet's Syndrome
Definition:

Behçet's (pronounced ‘BAY-sets') disease is a disease that causes symptoms in various parts of the body. It produces a group of symptoms that affect different body systems, including gastrointestinal, musculoskeletal, and the central nervous system. These symptoms include mouth or genital ulcers, skin lesions, and inflammation of the uvea which is an area around the pupil of the eye. Common symptoms include sores found in the mouth and on the genitals. More serious symptoms can include inflammation (combined with swelling, heat, redness, and pain) in the eyes and other parts of the body.


Prevalence:

Behçet’s syndrome is thought to be more commonly found along the ancient Silk Road, an area extending from Asia to the Mediterranean. Estimates from Turkey differ from 80-370 cases per 100,000 population, while prevalence estimates from East Asia (Japan, Korea, China) and the Middle East vary from 13-20 cases per 100,000 population. In the north of Spain, prevalence has been reported as 0.66 cases per 100,000 population, while the estimates from Germany are 2.26 cases per 100,000 population.


Symptoms and Signs:

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


Causes:

The causes of this syndrome are unknown. It is thought to be caused by a combination of hereditary and environmental factors. The HLA-B51 allele, which is one of the split antigens of B5, is commonly found in Asian and Middle Eastern patients, yet it is rare to find in northern European and North American patients. Infections may play a pathogenic role, as people who have Behçet's syndrome generally have a much higher incidence of antibodies to herpes simplex virus, hepatitis C virus, and parvovirus B19. Streptococcal antigens have also been implicated; a trial of prophylactic penicillin treatment was found to decrease the number of acute arthritis episodes in patients with Behçet's syndrome. Nearly all of the symptoms of Behçet's disease are due to blood vessel inflammation. Doctors aren't quite sure what causes this inflammation. It is thought that some people may have a problem with the immune system that gives them a higher chance to develop the disease. For these people, contact with substances in the environment, such as bacteria or viruses, may cause their immune system to attack the blood vessels. Behçet's disease is not contagious and will not spread from one person to another.


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behcetssyndrome



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