Thursday, October 18, 2007

Behcet's 101

Evidence of Behcet's Disease has existed for centuries. But it was not 'discovered' until 1937 when Turkish skin specialist, Professor Hulusi Behcet, published a report describing patients with recurrent mouth ulcers, genital ulcers and eye inflammations that the combination became known as 'Behcet's Disease'. Since this original description, however, people have come to believe that the disease may be associated with many other symptoms (gastrointestinal, pulmonary, cardiac, central nervous system ~~ in fact, wherever inflammation of the blood vessels occurs).
Causes for Behçet's are unknown; it is not known if there is just one cause. Whatever the cause, the result is that affected tissues become inflamed. Small blood vessels, particularly, become inflamed (this is known as vasculitis) and this can cause symptoms in many different parts of the body.
It is possible that a specific virus or bacterium may be the cause of the syndrome, but no specific infection has yet been clearly identified.
Most writers state with certainty that Behçet's is an autoimmune disease in which the immune system attacks the body's own tissues, but for others this is not yet certain; the disease is different from other autoimmune diseases in some aspects.
There is no evidence that Behçet's syndrome can be passed on to other people. It is not associated with cancer, or with a specific diet, or with any particular lifestyle (past or present). Most people with the syndrome will live a normal lifespan.
How common is Behçet's syndrome?
Behçet's syndrome is rare in the UK -- probably about 2,000 people are affected. In the US there may be 15,000 people affected by Behcet's.
The condition is more common in Mediterranean countries, Turkey, the Middle East, Japan and south-east Asia. Because Behçet's syndrome is more common in these regions, the illness has been called the Silk Route Disease, after the ancient trade route for silk which ran through these areas.
Behçet's can occur in most ethnic groups, and it is still unknown to what extent ethnic background increases or reduces risk. Behçet's occurs in males and females, and can affect all age groups, although it usually develops in the 20s and 30s. Some writers/researchers have said that males are more seriously/aggressively affected than males.
It is a long-term (chronic) problem which sometimes becomes less troublesome in later life.
Mouth ulcers occur in 97% of patients with Behcet's.
Genital ulceration and skin lesions occur in 75% or more of patients.
Brain (neurologic) disease occurs in less than 20% of patients.
Inflammation inside the eye occurs in up to 75 % of patients.
Both the small and large blood vessels (the vascular system) can be affected.
People who have vascular involvement are at an increased risk of a blood clot in a vein.
The vessels of the central nervous system brain, spinal cord, or heart may be affected.

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