of Behcet's disease. Inflammation of superficial veins that lie just under the skin can cause them to clot and form painful cords that can be felt (superficial thrombophlebitis). inflammation of larger, deep veins increases the risk of deep vein thrombosis. inflammation of arteries can lead to formation of aneurysms or to narrowing and blockage of an artery. if the blood flow to an organ is not adequate to supply oxygen and nutrients to the organ, then gangrene and death of tissue will occur.
There is no specific test for Behcet's disease, and the diagnosis is based on the combination of symptoms and disease manifestations. Diagnostic criteria, such as those set by the International Study Group for Behcet's disease in 1990, define criteria that groups of experts have generally agreed are needed to make the diagnosis. According to these criteria, recurrent mouth ulcers must be present with at least another two of the following symptoms: Recurrent genital ulcers, eye inflammation, a rash that is typical of Behcet's disease, or a positive pathergy test. The pathergy test is positive when pustules form around areas of minor skin trauma, for example at sites where the skin has been punctured with a needle to draw a blood sample. A test for pathergy is done by pricking the skin on the forearm with a sterile needle and seeing if a pustule forms at the puncture site. These diagnostic criteria have been established mainly for research protocols to make sure that research studies of patients with Behcet's disease are all discussing the same disease. The diagnostic criteria are not always helpful for an individual patient and do not have the same accuracy in different populations. For example, a positive pathergy test is common in Asian and Turkish patients but is less common in American patients.
The treatment of Behcet's disease depends on the organs involved and the severity of disease. if the gut, nervous system, or eyes are involved, the illness is treated aggressively with corticosteroids, usually prednisone, often combined with other drugs. colchicine and topical corticosteroids may also be helpful for oral and genital ulcers and eye inflammation. if these drugs do not control Behcet's disease, then immunosuppressants such as CHLORAMBUCIL, AZATHIOPRINE, METHOTREXATE, cyclophosphamide, and cyclosporine are added. There are good studies showing that azathioprine is effective, but there are a few controlled trials comparing different treatments. thalidomide is moderately effective for decreasing the frequency of attacks of oral and genital ulcers but has many side effects and is absolutely contraindicated if there is any chance of the patient becoming pregnant. Recently, alpha-interferon and tumor necrosis factor (tnf) antagonists have shown promise in early studies, but it is too early to tell what their role will be. if venous or arterial thrombosis occur, then anticoagulation, first with heparin and then with warfarin, is used.
Behcet's disease often has periods when the disease is inactive, interspersed with periods when it flares. complications include blindness, perforation of bowel wall, deep vein thrombosis, rupture of an aneurysm, and neurological damage, but death due to Behcet's is unusual.
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