Behçet’s disease: global epidemiology of an Old Silk Road disease

Behçet’s disease is a multisystem disorder named after the Turkish dermatologist, Hulusi Behçet (1889–1948), who recognised and reported in 1937 a triad of symptoms consisting of recurrent eye inflammation, oral ulcers and genital ulcers.1 The disease had also been alluded to in descriptions by physicians as far back as Hippocrates.2 Behçet’s disease is particularly common in the Far East and the Mediterranean basin, and is frequently noted between the 30th and 45th degree latitudes in Asian and European populations,3 corresponding to the Old Silk Road, an ancient trading route stretching between the Mediterranean, the Middle East and the Far East. In contrast, this disorder is uncommon in the American continents, Oceania and sub-Saharan Africa.3

The underlying pathological process of Behçet’s disease is a systemic occlusive vasculitis,4 resulting in the four major clinical manifestations of (1) recurrent and chronic intraocular inflammation, (2) recurrent aphthous ulcers of the mouth, (3) genital ulcers and (4) skin lesions that may include erythema nodosum, acneiform lesions and cutaneous hypersensitivity thrombophlebitis. The frequency of ocular involvement in Behçet’s disease is approximately 70%4 and is characterised by unilateral or bilateral acute episodes of iridocyclitis with or without hypopyon, and/or panuveitis.5^–7 The …