AbstractBehçet’s Disease (BD) was first described by Hulusi Behçet in 1937 as a disease characterized by recurrent aphthous ulceration in the mouth and genital area and hypopyon iridocyclitis. Diagnostic criteria developed by the International Working Group in 1990 were generally accepted among dermatologists. BD usually begins with oral ulcers, and the development of other systemic manifestations last for years. The disease often starts in the second and third decades, but a period of 1-8 years is required for a full clinical picture to be completed. Mucocutaneous findings are the most common manifestations during the course of BD. BD begins with mucocutaneous lesions in 70-95% of the patients. Despite the possibility to affect almost all systems, the disease is often accompanied by mucocutaneous findings. BD is a chronic, multisystemic disease that shows remissions and exacerbations during the course of the disease. In the last two decades, great progress has been made in the treatment of mucocutaneous and ocular involvement of BD. On the other hand, CNS involvement, treatment of thrombosis and arterial aneurysms of major vessels remains a problem. In this article knowledge about etiology and clinical features, has been reviewed.
Behçet H. Über rezidivierende, aphtöse, durch ein virus verursachte geschwüre am mund, am auge und an den genitalien. Dermatol Wochenschr 1937;105:1152-7.
Feigenbaum A. Description of Behçet's syndrome in the Hippocratic third book of endemic diseases. Br J Ophthalmol 1956;40:355-7.
Barnes CG. Behçet’s syndrome –classification criteria. Ann Med Interne 1999;150:477-82.
Saylan T. Life story of Dr. Hulusi Behçet. Yonsei Med J 1997;38:327-32.
Tüzün Y. Hulusi Behçet, MD. February 20, 1889 to March 8, 1948. Clin Dermatol 2006; 24:548-50.
Sut N, Seyahi E, Yurdakul S, Senocak M, Yazici H. A cost analysis of Behcet's syndrome in Turkey. Rheumatology (Oxford). 2007;46:678-82.
Hegab S, Al-Mutawa S. Immunopathogenesis of Behçet's disease. Clin Immunol 200;96:174-86.
Tüzün Y, Yurdakul S, Mat C, Özyazgan Y, Hamuryudan V, Tüzün B, Yazıcı H. Epidemiology of Behçet’s syndrome in Turkey. Int J Dermatol 1996;35:618-20.
Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med 1999;341:1284-91.
Önder M, Gürer MA. Behçet’s disease: An enigmatic vasculitis. Clin Dermatol 1999;17:571-6.
Jorizzo JL, Behçet’s Disease: Fitzpatrick’s Dermatology in General Medicine. Beşinci baskı. Freedberg IM, Eisen AZ, Wolf K, Austen KF, Goldsmith AL, Katz IS, Fitzpatrick TB (eds), McGraw-Hill Inc, New York 1999, S:2161-5.
Kaklamani VG, Kaklamanis PG. Treatment of Behçet's disease-an update. Semin Arthritis Rheum 2001;30:299-312.
Yazıcı H, Tüzün Y, Pazarlı H, Yurdakul S, Özyazgan Y, Özdoğan H, Serdaroğlu S, Ersanlı M, Ülkü BY, Müftüoğlu AÜ. Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis 1984;43:783-9.
Ghate JV, Jorizzo JL. Behçet's disease and complex aphthosis. J Am Acad Dermatol 1999;40:1-18.
Tursen U, Gürler A, Boyvat A. Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease. Int J Dermatol 2003;42:346-51.
Mazzoccoli G, Matarangolo A, Rubino R, Inglese M, De Cata A. Behçet syndrome: from pathogenesis to novel therapies. Clin Exp Med2016;16:1-12.
Gül A. Behçet's disease: an update on the pathogenesis. Clin Exp Rheumatol 2001;19(5 Suppl 24):6-12.
Azizlerli G, Aksungur VL, Sarıca R, Akyol E, Övül C. The association of HLA-B5 antigen with specific manifestations of Behçet's disease. Dermatology 1994;188:293-5.
Verity DH, Marr JE, Ohno S, Wallace GR, Stanford MR. Behçet's disease, the Silk Road and HLA-B51:historical and geographical perspectives. Tissue Antigens 1999;54:213-20.
Direşkeneli H. Behçet’s disease: infectous aetiology, new autoantigens and HLA-B51. Ann Rheum Dis 2001;60:996-1002.
Lehner T. Immunopathogenesis of Behçet’s disease. Ann Med Interne 1999;150:483-7.
Kaya Tİ, Dur H, Tursen U, Gürler A.Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease. Clin Exp Dermatol 2002;27:498-501.
Schirmer M, Calamia KT, Direskeneli H. Ninth International Conference on Behçet's Disease, Seoul, Korea, May 27-29, 2000. J Rheumatol 2001;28:636-9.
Alpsoy E. Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions. J Dermatol 2016;43:620-32.
Bang D. Clinical spectrum of Behçet's disease. J Dermatol 2001;28:610-3.
Çalgüneri M, Kiraz S, Ertenli I, Benekli M, Karaarslan Y, Çelik I. The effect of prophylactic penicillin treatment on the course of arthritis episodes in patients with Behçet's disease. A randomized clinical trial. Arthritis Rheum 1996;39:2062-5.
Mendes D, Correia M, Barbedo M, Vaio T, Mota M, Gonçalves O, Valente J. Behçet's disease-a contemporary review. J Autoimmun 2009;32:178-88.
Şahin S, Akoğlu T, Direşkeneli H, Sen LS, Lawrence R. Neutrophil adhesion to endothelial cells and factors affecting adhesion in patients with Behçet's disease. Ann Rheum Dis 1996;55:128-33.
Bank I, Duvdevani M, Livneh A. Expansion of gamma delta T-cells in Behçet's disease: Role of disease activity and microbial flora in oral ulcers. J Lab Clin Med 2003;141:33-40.
Turan B, Gallati H, Erdi H, Gürler A, Michel BA, Villiger PM. Systemic levels of the T cell regulatory cytokines IL-10 and IL-12 in Behçet's disease; soluble TNFR-75 as a biological marker of disease activity. J Rheumatol 1997;24:128-32.
Bayraktar Y, Özaslan E, Van Thiel DH. Gastrointestinal manifestations of Behçet's disease. J Clin Gastroenterol 2000;30:144-54.
Michelson JB, Friedlander MH.Behçet’s disease. Int J Ophthalmol 1990;30:271-8.
Akmaz Ö, Erel A, Gürer MA: Comparison of histopathologic and clinical evaluations of pathergy test in Behçet's disease. Int J Dermatol 2000;39:121-5.
Fresko İ, Yurdakul S, Hamuryudan V, Özyazgan Y, Mat C, Tanverdi MM, Yazıcı H. The management of Behçet’s syndrome. Ann Med Interne 1999;150:576-81.
Koşar A, Öztürk M, Haznedaroğlu İC, Karaaslan Y. Hemostatic parameters in Behçet’s disease:a reappraisal . Rheumatol Int 2002;22:9-15.
Lee YJ, Kang SW, Yang JI, Choi YM, Sheen D, Lee EB, et al. Coagulation parameters and plasma total homocystein levels in Behçet’s disease. Thrombosis Research 2002;106:19-24.
Yazici H, Tuzun Y, Pazarli H, Yurdakul S, Ozyazgan Y, Ozdoğan H, Serdaroğlu S, et al. Influence of age of onset and patient's sex on the prevalence and severity of manifestations of Behcet's syndrome. Ann Rheum Dis 1984;43:783-9.
Kurokawa MS, Suzuki N. Behcet’s disease. Clin Exp Med 2004;3:10-20.
Chang HK, Cheon KS. The clinical significance of a pathergy reaction in patients with Behcet's disease. J Korean Med Sci 2002;17:371-4.
Davatchi F, Akbaran M, Shahram F, Jamshidi A, Gharibdoost F, Chams C. Iran Behcet’s Disease Dynamic Activity Measure. Abstracts of the XIIth European Congress of Rheumatology. Hung Rheumatol Suppl 1991;32:10-100.
Yosipovitch G, Shohat B, Bshara J, Wysenbeek A, Weinberger A. Elevated serum interleukin 1 receptors and interleukin 1B in patients with Behcet's disease: correlations with disease activity and severity. Isr J Med Sci 1995;31:345-8.
Krause I, Rosen Y, Kaplan I, Milo G, Guedj D, Molad Y, et al. Recurrent aphthous stomatitis in Behcet's disease: clinical features and correlation with systemic disease expression and severity. J Oral Pathol Med 1999;28:193-6.
France B, Davatchi F, Mizushima Y, Hazma M, Dilşen N, Kansu E, et al. Criteria for diagnosis of Behçet’s disease. Lancet 1990;335:1078-80.
Tunç R, Uluhan A, Melikoğlu M, Özyazgan Y, Özdoğan H, Yazıcı H. A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome. Clin Exp Rheumatol 2001;19 (5 Suppl 24):45-7.
Calamia KT, Schirmer M, O’Duffy JD. Reply: Diagnostic criteria for Behçet's disease. J Rheumatol 2000;27:2049-50.
Fresko İ. Highlights of the 10th International Congress on Behçet's Disease. Clin Exp Rheumatol 2002;20:59-64.
Rotondo C, Lopalco G, Iannone F, Vitale A, Talarico R, Galeazzi M, et al. Mucocutaneous Involvement in Behçet's Disease: How Systemic Treatment Has Changed in the Last Decades and Future Perspectives. Mediators Inflamm 2015;2015:451675.
Alpsoy E, Akman A. Behçet's disease: an algorithmic approach to its treatment. Arch Dermatol Res 2009; 301:693-702.
Chams-Davatchi C, Barikbin B, Shahram F, Nadji A, Moghaddassi M, Yousefi M, et al. Pimecrolimus versus placebo in genital aphthous ulcers of Behcet's disease: a randomized double-blind controlled trial. Int J Rheum Dis 2010;13:253-8.
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