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Adamantiades-Behçet disease (ABD) is a chronic multisystemic vasculitis that is able to affect any human organ or system Recurrent oral ulcers are a very important clinical sign. ABD is a worldwide pathology, which prevalence varies according to the population and geographic location. Although ABD has been known for ages, its aetiology remains an enigma. Genetic, immunological and microbiological factors have been associated. A wide spectrum of clinical manifestations (oral, genital, cutaneous, ocular, neurological, vascular and gastrointestinal) and an unpredictable evolution with repeated periods of exacerbation and remission are the most representative aspects of this pathology. The complex treatment of ABD requires a deep multidisciplinary cooperation; therefore, there is an extensive development of new therapeutic agents that have improved the prognosis of ABD. In this review were analysed the main etiological, clinical and therapeutic aspects of the disease.
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