Beh？et disease is a chronic relapsing vasculitis with unclear etiology and immunopathogenesis. Antigenic stimuli, antigen presenting cells, T cells, monocyte, and neutrophil and endothelial cells are major parts of the pathology of the disease. Understanding of the new pathogenic mechanisms based on molecular structure of the disease helps us in improving the novel therapeutic modalities. These drugs target specific and nonspecific inhibition of the immun system. These therapies include biologic agents, new topical and systemic immunosuppressants, tolerizing agents, and immunoablation. Novel treatment will be promising to treat the especially recalcitrant cases to conventional therapy. In this paper, new aspect of the immunopathogenesis of Beh？et’s diseases and novel treatment modalities will be discussed. 1. Introduction Beh？et disease (BD) is a vasculitis that, characterized by recurrent aphthous stomatitis, genital ulcers, skin lesions, relapsing uveitis, articular, neurologic, urogenital, vascular, intestinal, and pulmonary manifestations [1–3]. BD has been reported worldwide but has a distinct geographic distrubition, with highest prevalences in countries such as Turkey, ？ran, and Japan which are place on silk road. Although much has been learned during recent years on the pathogenesis and treatment of the disease, the etiology and pathogenesis of BD have not been fully clarified [4, 5]. Symptoms of the disease are considered to be based on the correlation between the genetic intrinsic factors and the triggering extrinsic factors, because more than 60% of BD patients are associated with HLA-B 51. Immune-mediated mechanisms play a major role in the pathogenesis of the disease, and inflammatory mediators are also involved [4–6]. Nowadays, recent investigations have made clear explanations about the pathogenesis of the disease. The hypersensitivity of T lymphocytes to different types of antigens plays a crucial role in the pathogenesis [3–6]. The present paper overviews an update on the immunopathogenesis of BD and also novel treatment based on pathogenesis. 2. Immunopathogenesis of Beh？et’s Disease BD is an inflammatory disorder characterized mainly by mucocutaneous findings and uveitis. However, it can be present with other cutaneous symptoms such as pseudofolliculitis, erythema nodosum, and pyoderma gangrenosum [2–5]. It can be present with articular, neurological, pulmonary, intestinal manifestations other than classical triad. The close relationship between the genetic and triggering external factors is thought to be present in the pathogenesis
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