Adamantiades–Behçet Disease

Abstract

Adamantiades–Behçet disease (ABD) is a rare, multisystem inflammatory disease of unknown aetiology, classified as a systemic vasculitis and as a neutrophilic dermatosis involving all types and sizes of blood vessels. A genetically determined background with environmental triggering factor(s) are probably involved. A chronic, relapsing, progressive course of oral aphthous ulcers, genital ulcers, skin lesions (papulopustules, erythema nodosum) and iridocyclitis/posterior uveitis is characteristic. ABD is occasionally accompanied by arthritis and vascular, neurological and gastrointestinal manifestations. ABD has a worldwide occurrence with varying prevalence, being endemic in eastern and central Asian and the eastern Mediterranean countries. Characteristic histopathological features of ABD are vasculitis and thrombosis. ABD is not considered contagious. There is no specific mode of Mendelian transmission in ABD. The disease has potentially poor prognosis (especially in males with systemic presenting signs). HLA‐B51 and ERAP‐1 seem to be associated with a more severe prognosis. Immunological mechanisms are considered to play a major role in the pathogenesis of ABD. The disease has currently been classified among the autoinflammatory disorders. The endothelium seems to be the primary target. Diagnosis of ABD is based on clinical signs. The Revised International Criteria for Behçet's Disease provides the most accurate diagnosis. Ophthalmic and neurological sequelae are leading causes of morbidity, followed by severe vascular and gastrointestinal manifestations. Their effects on morbidity may be cumulative. The clinical course of ABD is variable. A multidisciplinary approach in the management of ABD patients is mandatory. The choice of treatment depends on the site and severity of the clinical manifestations of the disease.