Adamantiades–Behçet disease is a multisystem inflammatory disease of unknown aetiology, classified as a systemic vasculitis and as a neutrophilic dermatosis involving all types and sizes of blood vessels. It is characterized clinically by recurrent courses of oral aphthous ulcers, genital ulcers, skin lesions (papulopustules, erythema nodosum) and iridocyclitis/posterior uveitis. It is occasionally accompanied by arthritis and vascular, neurological, gastrointestinal or other manifestations. It is a rare disease with worldwide distribution but strongly varying prevalence; certain ethnic groups are mainly affected. It exhibits a genetically determined background with environmental triggering factor(s) probably involved. Diagnosis is defined by new clinical criteria. It presents a chronic, relapsing, progressive course and potentially poor prognosis (especially in males with systemic presenting signs).
Keywords Adamantiades–Behçet disease, Behçet disease, Behçet syndrome, malignant aphthosis, epidemiology, pathophysiology, treatment