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BackgroundBehçet’s disease (BD) is a chronic and recurrent inflammatory disease of unknown etiology, classified into polygenic autoinflammatory diseases or variable vessel vasculitis. It has a wide spectrum of symptoms with a very variable range of severity, from mucocutaneous involvement to neurological manifestations, systemic vascular or severe ocular manifestations. About 5.4%–7.6% of Behçet’s cases have a paediatric debut.ObjectivesTo evaluate and compare the clinical and laboratory manifestations of a series of 52 patients, adults and children, diagnosed with BD according to the classification criteria of the International Study Group of BD (ISGBD-1990).MethodsRetrospective cross-sectional observational study, which included 43 adult patients and 9 paediatric patients diagnosed with EB in the Rheumatology Department of a Madrid tertiary hospital. The clinical-analytical characteristics of both groups were evaluated, as well as the correlation of HLA-B51 with the described symptomatology.ResultsThe mean age at diagnosis of BD was 36.9±11.8 years in adults and 11.4±5.1 years in children. 27.3% of adults and 11.1% of children with BD were male, with oral ulcers close to 90% in both groups. Contrary to what was reported in other series, genital ulcers were more frequent in children (77.8% versus 65.9% of adults), as was the presence of uveitis (44.4% in children compared to 22.7% in adults) and neurological manifestations (22.2% in children versus 6.8% in adults). Joint involvement was also more frequent in children (88.9% versus 52.3% in adults), as well as fever (44% in children versus 14% in adults); being these two manifestations the only parameters that were associated in a statistically significant way with their presentation in the paediatric age in BD. In contrast, skin involvement and vascular manifestations were more frequent in adults. The positivity of HLA-B51 did not correlate statistically with any clinical manifestation, but those who had it had a mean age at diagnosis of 26.5 years compared to a mean of 39 years in those who did not present this genetic marker.ConclusionsBehçet’s disease presents with a wide spectrum of clinical manifestations, potentially serious, ranging from skin lesions to neurological or vascular manifestations. In our series, patients diagnosed at paediatric age most frequently had systemic manifestations (fever), arthritis or severe clinical manifestations such as neurological involvement or uveitis. Limitations: a small number of paediatric cases included in our study.Disclosure of InterestNone declared