Behcet's disease is characterized by all of the following Except
High-Yield Explanation
Behcet's disease is a clinicopathologic entity characterized by recurrent episodes of oral and genital ulcers, iritis, and cutaneous lesions. The underlying pathologic process is a leukocytoclastic venulitis, although vessels of any size and in any organ can be involved.The recurrent aphthous ulcerations are a sine qua non for the diagnosis.Skin involvement is observed in 80% of patients and includes folliculitis, erythema nodosum, an acne-like exanthem, and, infrequently, vasculitis, Sweet syndrome, and pyoderma gangrenosum. Nonspecific skin inflammatory reactivity to any scratches or intradermal saline injection (pathergy test) is a common and specific manifestation.Eye involvement with scarring and bilateral panuveitis is the most dreaded complication since it occasionally progresses rapidly to blindness. In addition to iritis, posterior uveitis, retinal vessel occlusions, and optic neuritis can be seen in some patients with the syndrome.Nondeforming ahritis or ahralgias are seen in 50% of patients and affect the knees and ankles.Superficial or deep peripheral vein thrombosis is seen in 30% of patients. Aerial involvement occurs in less than 5% of patients and presents with aoitis or peripheral aerial aneurysm and aerial thrombosis.Mucous membrane involvement may respond to topical glucocoicoids in the form of mouthwash or paste. In more serious cases, thalidomide (100 mg/d) is effective.Colchicine can be beneficial for the mucocutaneous manifestations and ahritis. Uveitis and CNS-Behcet's syndrome require systemic glucocoicoid therapy (prednisone, 1mg/kg per day) and azathioprine (2-3 mg/kg per day). Cyclosporine (5 mg/kg) has been used for sight-threatening uveitis, alone or in combination with azathioprine.Ref: Harrison; 19th edition; Page no: 2194