RESUMO
Ten patients (seven males and three females) with the diagnostic criteria for Behçet's disease are reported. Symptoms began between ages 14 and 37 (mean age 27.9 years), and the time of evolution of the disease varied between one and 23 years (mean 5.7 years). Initial presentation was aphthous stomatitis (three cases), ocular inflammation (three cases), erythema nodosum (one case), arthritis (one case), thrombophlebitis (one case), and hemorrhagic proctocolitis (one case). During the course of the disease the commonest clinical manifestations were oral and genital ulcerations (100%) and ocular (70%), cutaneous (70%), articular (40%), neurological (40%), venous (40%), gastrointestinal (40%) and arterial (10%) involvement. Increased erythrocyte sedimentation rate was a constant feature in all patients. HLA-B5 antigen was determined in four patients, being positive in two of them. Cerebrospinal fluid alterations were present in the four patients with neurological involvement even though three of them never had meningeal symptoms. One patient developed portal hypertension secondary to portal vein thrombosis, and its clinical manifestations are described.