Behçet’s Disease: An Enigmatic Malady with Plethoric Expressions

Abstract

Behçet's disease (BD) is a chronic, inflammatory, repetitive, debilitating systemic vasculitis with conglomerate system involvement of uncharted aetiology portrayed by the triad of oral ulcers, genital ulcers and cutaneous lesions. Behçet's disease has a universal distribution with a predominance among populace with higher prevalence of human leukocyte antigen (HLA) B5 and its split, HLA-B51, in the Mediterranean basin, the Middle East and Far East. This ailment is presently acknowledged to be a multisystem disorder with mucocutaneous, ocular, intestinal, articular, vascular, urogenital, musculoskeletal, cardiopulmonary and neurologic systems and hence bears significant organ sinistering morbidity and mortality. The diagnosis of Behçet's disease relies on scrupulous history, identification of its emblematic clinical countenance as per the diagnostic criteria construed by the International Study Group (ISG) and examination of all pertinent systems and excluding other systemic rheumatic diseases. However, there is a lag in diagnosis because of its plethoric presentations and a dearth of specific biomarkers. The multiple organ and tissue involvement of Behçet's disease necessitates an interdisciplinary approach from various health specialities since a general and an organspecific approach is mandatory. Prime concern is to control the active periods of the disease which is achieved by different anti-inflammatory drugs, steroids or immunomodulators.