Behçet's syndrome

Behçet's syndrome (BS) is a rare systemic vasculitis that can involve the skin, mucosa, joints, vessels, eyes, nervous and gastrointestinal systems. These manifestations can present alone or coexist in the same patient in specific clusters that can require different treatment strategies. BS is a ubiquitous disease although it has a peculiar geographical distribution, being almost endemic in Eurasian countries. The aetiopathogenesis of BS is still unknown and probably depends on the interaction of different pathways, including genetic predisposition, immune dysregulation and infectious triggers. The therapeutic approach includes systemic corticosteroids, colchicine for mild forms of BS, and traditional or biological immunosuppressive agents for moderate to severe disease. Here we summarize the clinical and therapeutical aspect of BS according to the most recent scientific evidence.