Titlebook: Beh?et‘s Syndrome; From Pathogenesis to Lorenzo Emmi Book 2014 Springer-Verlag Italia 2014 Autoimmune disease.Autoinflammatory disease.Beh?

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Rare Diseases of the Immune Systemhttp://image.papertrans.cn/b/image/182955.jpg

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,The Numbers of Beh?et: A Rare Disease?, rare disease on an estimated total number of rare conditions of about 6,000 to 8,000. Numbers of Beh?et disease (.) reveals significant regional differences. The highest prevalence rates are recorded in the Mediterranean and Middle/Far Eastern regions (e.g. in Turkey it affects 400:10,000 individua

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,From Hippocrates to Hulusi Beh?et: What History, first recognized by Hulusi Beh?et (1889–1948) with a patient in 1924, but the history of this disease is supposed to date back to the fifth century BC, when Hippocrates reported some individual indicators, attributed to an originally endemic and epidemic disease. Due to the tricky manifestations of

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,Infections, Autoimmunity, and Beh?et’s Syndrome: What Liaison?,ferent microorganisms, specialized types of specific responses allow the recognition and elimination of infectious agents. All individuals harbor autoreactive T cells that need activation and critical expansion in order to start active autoimmune disease. In a number of human diseases and in their c

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,Pathogenesis of Beh?et Syndrome,s, skin lesions, and less often central nervous system (CNS) and/or vascular manifestations. Despite the amount of new data accumulated during the recent years, the pathogenetic mechanisms underlying BS still remain partially unknown; however, the interaction between a specific genetic background an

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,Mucocutaneous Involvement in Beh?et’s Syndrome,ar in patients with BD. They present as orogenital ulcers, papulopustular eruptions, erythema nodosum, superficial thrombophlebitis, necrotizing vasculitis, and pyoderma gangrenosum. All patients with orogenital ulcerations, papulopustular eruptions, and thrombophlebitis should be fully investigated

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,Articular and Muscular Manifestations in Beh?et’s Disease,f BD in 11–34?% and may appear several years before other symptoms. Knees, wrists, ankles, and elbows are more often affected while the involvement of the small joints of hands and feet is less common. Arthralgia is the main symptoms in 80?% of patients while arthritis is less common and includes mo