The aim of this study was to determine the demographic and clinical characteristics in patients diagnosed with Beh double dagger et's disease (BD) in Brazil. We performed a retrospective review of all the patients' records with BD diagnosed from 1988 to 2010 in the Rheumatology Department at the State University of Campinas (UNICAMP). All patients had to fulfill the International Study Group for Beh double dagger et's disease diagnostic criteria. Eighty-seven patients were included in the study. The female/male ratio was 1.18:1, and the mean age at the onset of the disease onset was 28.03 +/- A 7.57 years. Oral aphthosis was the most frequent manifestation (100%). Genital aphthosis was also frequent (77%), followed by pseudofolliculitis (47.67%). Ocular symptoms were present in 80% and neurological manifestations in 31.03% of the patients. Arthralgia was reported in 31.03% and arthritis in 13.79% of the cases. Vascular involvement was seen in 13.95% of the patients. Only 1.14% had gastrointestinal involvement. This series, from a South American country, showed a similar general pattern of the BD to those found in different endemic areas in the world, with a high frequency of ocular and neurological manifestations.32720632067