Behcet's syndrome

Behcet's syndrome is a vasculitis with mucocutaneous, ophthalmological, vascular, gastrointestinal, and central nervous system (CNS) manifestations.

Most commonly seen in young people aged 20-30 years.

More active and severe in men.

Azathioprine and other immunosuppressive therapies are used when severe manifestations such as eye, vascular, and CNS involvement are present.

In many patients, disease activity decreases with time, and this natural course can affect treatment decisions about the type and duration of medical therapy.

Behcet's syndrome, a systemic vasculitis first described by Hulusi Behcet - a Turkish dermatologist - is most commonly seen in Turkey, Israel, the Mediterranean basin, and eastern Asia (where Japan and South Korea report the most cases).​[1]Behcet H. Uber rezidivierende aphthöse, durch ein Virus Verursachte Geschwure am Mund, am Auge und an den Genitalien. Dermatol Wschr [in German]. 1937;105:1152-7.​ It can cause skin and mucosal lesions, uveitis, major arterial and venous vessel disease, and gastrointestinal and neurological manifestations. These can present in various combinations and sequences in patients over time.​[2]Yazici H, Seyahi E, Hatemi G, et al. Behçet syndrome: a contemporary view. Nat Rev Rheumatol. 2018 Feb;14(2):107-19. http://www.ncbi.nlm.nih.gov/pubmed/29296024?tool=bestpractice.com ​