What is Behçet's disease?
Behçet’s disease, or Behçet’s syndrome, usually appears in early adulthood with painful aphthous ulcers (similar to canker sores) in the mouth and genitals. These ulcers generally subside after one to two weeks, but they recur throughout the course of the disease. Some patients also have painful skin nodules known as erythema nodosum. Brief periods of joint pain are also common. Rarely, the ulcers of Behçet’s disease are found in the gastrointestinal tract. Involvement of the central nervous system causes a variety of symptoms ranging from mild confusion to paralysis. However, the most serious complication of Behçet’s disease is involvement of the eyes, since this may progress to blindness. Patients also have an increased predisposition for the formation of blood clots, usually in the venous system. These clots can be life-threatening if they migrate to the lungs.
The common factor underlying these diverse manifestations of Behçet’s disease is inflammation of the small blood vessels, but it remains unclear what environmental or genetic factors trigger this inflammation. Rare reports have been made of familial forms of the disease, raising the possibility of a genetic basis, but this remains under investigation. Diagnosis is made on clinical grounds.
Behçet’s disease is a chronic condition, but the severity of the disease generally lessens over time. Ulcers are first treated with topical glucocorticoids, in the form of either mouthwash or paste. Refractory cases may require thalidomide, a drug that must be used with extreme caution because of its ability to cause birth defects. Because of the risk of blindness, ocular and central nervous system disease is managed more aggressively with oral steroids (such as prednisone), along with azathioprine or cyclosporine.
Apart from the above complications, the long-term prognosis of Behçet’s disease is quite good, and the life expectancy of patients is comparable with that of the general population.
The first description of Behçet’s disease appears in the writings of Hippocrates in the fifth century b.c.e., but it was first recognized in the modern era by the Turkish physician Hulusi Behçet in 1937. Although Behçet’s disease is seen throughout the world, the highest prevalence is in countries along the ancient Silk Road, a trading route extending from the Far East to the Mediterranean Sea.
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