Behçet disease

Abstract

Behçet disease is a multisystem disorder characterized by relapsing inflammation of unknown origin. The disease has first been described as a distinct clinical entity by a Turkish dermatologist, Prof. Hulusi Behçet, as a triple symptom complex of oral and genital ulcers and hypopyon iritis in 1937. The disease is now accepted as an obliterative and necrotizing systemic vasculitis involving different organ systems and leading to a wide range of clinical manifestations. The usual course of the disease is characterized by recurrent inflammatory episodes. The eye is the most commonly involved vital organ. Uveitis associated with Behçet disease, which will be referred to as Behçet uveitis throughout this chapter, represents one of the most difficult forms of uveitis to treat. Despite the use of robust medical therapy with corticosteroids and conventional immunomodulatory and biologic agents, some patients with Behçet uveitis still end up with poor vision, and Behçet uveitis remains as a potentially blinding disease.