Behcet's disease

Behcet's (beh-CHETS) disease, also called Behcet's syndrome, is a rare disorder that causes blood vessel inflammation throughout your body. The disease can lead to numerous signs and symptoms that can seem unrelated at first. They can include mouth sores, eye inflammation, skin rashes and lesions, and genital sores. Treatment involves medications to reduce the signs and symptoms of Behcet's disease and to prevent serious complications, such as blindness.

Behcet's disease symptoms vary from person to person, can come and go or become less severe over time. Signs and symptoms depend on which parts of your body are affected. Areas commonly affected by Behcet's disease include:

Mouth – Painful mouth sores that look similar to canker sores are the most common sign of Behcet's disease. They begin as raised, round lesions in the mouth that quickly turn into painful ulcers. The sores usually heal in one to three weeks, though they do recur.

Skin – Some people develop acne-like sores on their bodies. Others develop red, raised and tender nodules on their skin, especially on the lower legs.

Genitals – Red, open sores can occur on the scrotum or the vulva. The sores are usually painful and can leave scars.

Eyes – Inflammation in the eye (uveitis) causes redness, pain and blurred vision, typically in both eyes. In people with Behcet's disease, the condition can come and go.

Joints – Joint swelling and pain often affect the knees in people with Behcet's disease. The ankles, elbows or wrists also might be involved. Signs and symptoms can last one to three weeks and go away on their own.

Blood vessels – Inflammation in veins and arteries can cause redness, pain, and swelling in the arms or legs when a blood clot results. Inflammation in the large arteries can lead to complications, such as aneurysms and narrowing or blockage of the vessel.

Digestive system – A variety of signs and symptoms can affect the digestive system, including abdominal pain, diarrhea and bleeding.

Brain – Inflammation in the brain and nervous system can cause headache, fever, disorientation, poor balance or stroke.

In 1937, Hulusi Behçet, a Turkish dermatologist, described a syndrome characterized by recurrent oral ulcers, genital ulcers, and hypopyon uveitis of unknown cause. The diverse range of clinical findings seems to stem from an underlying vasculitis; however, the ultimate etiologic agent remains unknown.

Occurring most frequently in the Middle East, Behçet's is endemic among populations clustered along the historic silk road, which extended from eastern Asia to the Mediterranean basin. Highest prevalence is in Iran, Turkey, and other Near Eastern or Mediterranean Basin countries. In the Middle East, Europe and the United States, young men are most often affected; however, in Japan and Korea there appears to be a slight female preponderance. Current prevalence is estimated to range from a low of 0.3 per 100,000 in Northern Europe to a peak of 16 to 100 per 100,000 in Iran.