Behcet’s syndrome (BS) is a perplexing multisystem inflammation which, in the most recent definition of diagnostic criteria, has one basic major feature-recurrent oral aphthous ulcers (AU)-and two of the following features: recurrent genital AU, eye lesions (posterior uveitis), skin lesions (erythema nodosum or pustules). Other manifestations include synovitis, neurologic disorders, and thrombophlebitis.
Causes of Behcet’s Syndrome
Etiology unknown. In the eastern Mediterranean and East Asia, HLA-B5 and HLA-B51 association: in the United States and Europe, no consistent HLA association, The lesions could be the result of an accumulation of neutrophils in the sites of immune complex-mediated vasculitis.
Symptoms of Behcet’s Syndrome
The symptoms of Behcet’s syndrome depend on the area of the body affected. Behcet’s syndrome can involve inflammation of many areas of the body. These areas include the arteries that supply blood to the body’s tissues. Behcet’s syndrome can also affect the veins that take the blood back to the lungs to replenish the oxygen content. Other areas of body that can be affected by the inflammation of Behcet’s syndrome include the back of the eyes (retina), brain, joints, skin, and bowels.
Inflammation of the eye, which can involve the front of the eye (uvea) causing uveitis, or the back of the eye (retina) causing retinitis, can lead to blindness. Symptoms of eye inflammation include pain, blurred vision, tearing, redness, and pain when looking at bright lights.
Symptoms of inflammation of the brain or tissue that covers the brain (meninges) include headaches, neck stiffness, and is often associated with fever.
Diagnosis
Proposed criteria for Behcet’s syndrome include the presence of oral AU plus two of the following: recurrent genital AU, eye lesions, skin lesions, or positive pathergy test.
Treatment
Aphthous Ulcers Potent topical glucocorticoids. Intralesional triamcinolone, 3 to 10 rng/ml, injected into ulcer base. Thalidomide, 100 mg PO bid. Colchicine, .6 mg PO 2 to 3 times a day. Dapsone.
Systemic Involvement Prednisone with or without azathioprine, cyclophosphamide, azathioprine alone, chlorambucil, cyclosporine.
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