Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Clinical manifestations and diagnosis of Behçet’s syndrome

Ellison L Smith, MD
Yusuf Yazici, MD
Section Editor
Peter A Merkel, MD, MPH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


Behçet’s syndrome is characterized by recurrent oral aphthae and any of several systemic manifestations including genital aphthae, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease, or arthritis. Behçet’s may have been described by Hippocrates, but it was brought to the attention of the modern medical community by Hulusi Behçet in 1937 [1-3].

Most clinical manifestations of Behçet’s syndrome are believed to be due to vasculitis. Among the systemic vasculitides, Behçet’s syndrome is remarkable for its ability to involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides of the circulation.

The clinical manifestations, diagnosis, and differential diagnosis of Behçet’s syndrome are reviewed here. The pathogenesis and treatment of this disorder are discussed separately. (See "Pathogenesis of Behçet’s syndrome" and "Treatment of Behçet’s syndrome".)


Behçet’s syndrome is more common (and often more severe) along the ancient silk road, which extends from eastern Asia to the Mediterranean [4,5]. It is most common in Turkey (80 to 370 cases per 100,000) while the prevalence ranges from 13.5 to 35 per 100,000 in Japan, Korea, China, Iran, Iraq, and Saudi Arabia [4]. By comparison, the prevalence ranges from 1 per 15,000 to 1 per 500,000 in North American (Olmsted County, Minnesota) and Northern European countries [6]. The prevalence is similar in men and women in the areas where it is more common, but women are affected more commonly in reports from the United States and northern Europe. It typically affects young adults 20 to 40 years of age but is infrequently also seen in children [7-9]. The disease appears to be more severe in young, male, and Middle- or Far-Eastern patients [10-16]. Most cases of Behçet’s are sporadic, although families with multiple affected members, which is known as familial clustering, have been reported, and having a first-degree relative with Behçet’s does increase risk for the disease [17,18]. Earlier onset of disease in successive generations, known as genetic anticipation, has been described [19].


The common clinical feature in patients with Behçet’s syndrome is the presence of recurrent and usually painful mucocutaneous ulcers. Other clinical manifestations of this disorder are more variable among different patients and populations.


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Sep 2016. | This topic last updated: Jul 7, 2015.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
  1. FEIGENBAUM A. Description of Behçet's syndrome in the Hippocratic third book of endemic diseases. Br J Ophthalmol 1956; 40:355.
  2. Behcet H. Uber rezidivierende, aphthose durch ein virus verursachte geschwure am mund, am auge und an der genitalen. Dermatologische Wochenschrift 1937; 105:1152.
  3. Mutlu S, Scully C. The person behind the eponym: Hulûsi Behçet (1889-1948). J Oral Pathol Med 1994; 23:289.
  4. Yurdakul S, Hamuryudan V, Yazici H. Behçet syndrome. Curr Opin Rheumatol 2004; 16:38.
  5. Yazici H, Fresko I, Yurdakul S. Behçet's syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol 2007; 3:148.
  6. Calamia KT, Wilson FC, Icen M, et al. Epidemiology and clinical characteristics of Behçet's disease in the US: a population-based study. Arthritis Rheum 2009; 61:600.
  7. Treudler R, Orfanos CE, Zouboulis CC. Twenty-eight cases of juvenile-onset Adamantiades-Behçet disease in Germany. Dermatology 1999; 199:15.
  8. Tugal-Tutkun I, Urgancioglu M. Childhood-onset uveitis in Behçet disease:a descriptive study of 36 cases. Am J Ophthalmol 2003; 136:1114.
  9. Karincaoglu Y, Borlu M, Toker SC, et al. Demographic and clinical properties of juvenile-onset Behçet's disease: A controlled multicenter study. J Am Acad Dermatol 2008; 58:579.
  10. Mok CC, Cheung TC, Ho CT, et al. Behçet's disease in southern Chinese patients. J Rheumatol 2002; 29:1689.
  11. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med 1999; 341:1284.
  12. O'Duffy JD. Behçet's disease. Curr Opin Rheumatol 1994; 6:39.
  13. Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 2003; 82:60.
  14. Salvarani C, Pipitone N, Catanoso MG, et al. Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: a seventeen-year population-based study. Arthritis Rheum 2007; 57:171.
  15. Olivieri I, Leccese P, Padula A, et al. High prevalence of Behçet's disease in southern Italy. Clin Exp Rheumatol 2013; 31:28.
  16. Savey L, Resche-Rigon M, Wechsler B, et al. Ethnicity and association with disease manifestations and mortality in Behçet's disease. Orphanet J Rare Dis 2014; 9:42.
  17. Akpolat T, Koç Y, Yeniay I, et al. Familial Behçet's disease. Eur J Med 1992; 1:391.
  18. Koné-Paut I, Geisler I, Wechsler B, et al. Familial aggregation in Behçet's disease: high frequency in siblings and parents of pediatric probands. J Pediatr 1999; 135:89.
  19. Gülbay B, Acican T, Erçen Diken Ö, Pinar Önen Z. Familial Behçet's disease of adult age: a report of 4 cases from a Behçet family. Intern Med 2012; 51:1609.
  20. Zouboulis CC, Vaiopoulos G, Marcomichelakis N, et al. Onset signs, clinical course, prognosis, treatment and outcome of adult patients with Adamantiades-Behçet's disease in Greece. Clin Exp Rheumatol 2003; 21:S19.
  21. Uluduz D, Kürtüncü M, Yapıcı Z, et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology 2011; 77:1900.
  22. O'Duffy JD. Behcet's syndrome. In: Primer on the Rheumatic Diseases, 10th, Arthritis Foundation, Atlanta 1993. Vol 29, p.206.
  23. Soy M, Erken E, Konca K, Ozbek S. Smoking and Behçet's disease. Clin Rheumatol 2000; 19:508.
  24. Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet 1990; 335:1078.
  25. Kaklamani VG, Vaiopoulos G, Markomichelakis N, Kaklamanis P. Recurrent epididymo-orchitis in patients with Behçet's disease. J Urol 2000; 163:487.
  26. Cho YH, Jung J, Lee KH, et al. Clinical features of patients with Behçet's disease and epididymitis. J Urol 2003; 170:1231.
  27. Demirkesen C, Tüzüner N, Mat C, et al. Clinicopathologic evaluation of nodular cutaneous lesions of Behçet syndrome. Am J Clin Pathol 2001; 116:341.
  28. Diri E, Mat C, Hamuryudan V, et al. Papulopustular skin lesions are seen more frequently in patients with Behçet's syndrome who have arthritis: a controlled and masked study. Ann Rheum Dis 2001; 60:1074.
  29. Tunc R, Keyman E, Melikoglu M, et al. Target organ associations in Turkish patients with Behçet's disease: a cross sectional study by exploratory factor analysis. J Rheumatol 2002; 29:2393.
  30. Hatemi G, Bahar H, Uysal S, et al. The pustular skin lesions in Behcet's syndrome are not sterile. Ann Rheum Dis 2004; 63:1450.
  31. Dinç A, Karaayvaz M, Caliskaner AZ, et al. Dermographism and atopy in patients with Behçet's disease. J Investig Allergol Clin Immunol 2000; 10:368.
  32. Lê Thi Huong D, Wechsler B, Papo T, et al. Arterial lesions in Behçet's disease. A study in 25 patients. J Rheumatol 1995; 22:2103.
  33. O'Duffy JD. Vasculitis in Behçet's disease. Rheum Dis Clin North Am 1990; 16:423.
  34. Vaiopoulos G, Pangratis N, Samarkos M, et al. Nailfold capillary abnormalities in Behçet's disease. J Rheumatol 1995; 22:1108.
  35. Movasat A, Shahram F, Carreira PE, et al. Nailfold capillaroscopy in Behçet's disease, analysis of 128 patients. Clin Rheumatol 2009; 28:603.
  36. Nussenblatt RB. Uveitis in Behçet's disease. Int Rev Immunol 1997; 14:67.
  37. Seyahi E, Melikoglu M, Yazici H. Clinical features and diagnosis of Behcet's syndrome. Int J Adv Rheumatol 2007; 5:8.
  38. Matsuo T, Itami M, Nakagawa H, Nagayama M. The incidence and pathology of conjunctival ulceration in Behçet's syndrome. Br J Ophthalmol 2002; 86:140.
  39. Zamir E, Bodaghi B, Tugal-Tutkun I, et al. Conjunctival ulcers in Behçet's disease. Ophthalmology 2003; 110:1137.
  40. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, et al. Neovascularization of the optic disc in Behçet's disease. Jpn J Ophthalmol 2006; 50:256.
  41. Tugal-Tutkun I, Onal S, Ozyazgan Y, et al. Validity and agreement of uveitis experts in interpretation of ocular photographs for diagnosis of Behçet uveitis. Ocul Immunol Inflamm 2014; 22:461.
  42. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, et al. Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol 2004; 138:373.
  43. Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. Brain 1999; 122 ( Pt 11):2171.
  44. Serdaroğlu P. Behçet's disease and the nervous system. J Neurol 1998; 245:197.
  45. Siva A, Saip S. The spectrum of nervous system involvement in Behçet's syndrome and its differential diagnosis. J Neurol 2009; 256:513.
  46. Al-Araji A, Kidd DP. Neuro-Behçet's disease: epidemiology, clinical characteristics, and management. Lancet Neurol 2009; 8:192.
  47. Atasoy HT, Tunc TO, Unal AE, et al. Peripheral nervous system involvement in patients with Behçet disease. Neurologist 2007; 13:225.
  48. Akbulut L, Gur G, Bodur H, et al. Peripheral neuropathy in Behçet disease: an electroneurophysiological study. Clin Rheumatol 2007; 26:1240.
  49. Benamour S, Naji T, Alaoui FZ, et al. [Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature]. Rev Neurol (Paris) 2006; 162:1084.
  50. Lee SH, Yoon PH, Park SJ, Kim DI. MRI findings in neuro-behçet's disease. Clin Radiol 2001; 56:485.
  51. Kalra S, Silman A, Akman-Demir G, et al. Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations. J Neurol 2014; 261:1662.
  52. Farah S, Al-Shubaili A, Montaser A, et al. Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations. J Neurol Neurosurg Psychiatry 1998; 64:382.
  53. Allen NB. Miscellaneous vasculitic syndromes including Behçet's disease and central nervous system vasculitis. Curr Opin Rheumatol 1993; 5:51.
  54. Saadoun D, Wechsler B, Resche-Rigon M, et al. Cerebral venous thrombosis in Behçet's disease. Arthritis Rheum 2009; 61:518.
  55. Hamuryudan V, Er T, Seyahi E, et al. Pulmonary artery aneurysms in Behçet syndrome. Am J Med 2004; 117:867.
  56. Sarica-Kucukoglu R, Akdag-Kose A, KayabalI M, et al. Vascular involvement in Behçet's disease: a retrospective analysis of 2319 cases. Int J Dermatol 2006; 45:919.
  57. Fei Y, Li X, Lin S, et al. Major vascular involvement in Behçet's disease: a retrospective study of 796 patients. Clin Rheumatol 2013; 32:845.
  58. Koç Y, Güllü I, Akpek G, et al. Vascular involvement in Behçet's disease. J Rheumatol 1992; 19:402.
  59. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behçet disease. Curr Opin Rheumatol 2005; 17:1.
  60. Uzun O, Akpolat T, Erkan L. Pulmonary vasculitis in behcet disease: a cumulative analysis. Chest 2005; 127:2243.
  61. Seyahi E, Melikoglu M, Akman C, et al. Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients. Medicine (Baltimore) 2012; 91:35.
  62. Erkan F. Pulmonary involvement in Behçet disease. Curr Opin Pulm Med 1999; 5:314.
  63. Hamuryudan V, Oz B, Tüzün H, Yazici H. The menacing pulmonary artery aneurysms of Behçet's syndrome. Clin Exp Rheumatol 2004; 22:S1.
  64. Erkan D, Yazici Y, Sanders A, et al. Is Hughes-Stovin syndrome Behçet's disease? Clin Exp Rheumatol 2004; 22:S64.
  65. Bayraktar Y, Balkanci F, Bayraktar M, Calguneri M. Budd-Chiari syndrome: a common complication of Behçet's disease. Am J Gastroenterol 1997; 92:858.
  66. Ames PR, Steuer A, Pap A, Denman AM. Thrombosis in Behçet's disease: a retrospective survey from a single UK centre. Rheumatology (Oxford) 2001; 40:652.
  67. Uzun O, Erkan L, Akpolat I, et al. Pulmonary involvement in Behçet's disease. Respiration 2008; 75:310.
  68. Kim HA, Choi KW, Song YW. Arthropathy in Behçet's disease. Scand J Rheumatol 1997; 26:125.
  69. Moses Alder N, Fisher M, Yazici Y. Behçet's syndrome patients have high levels of functional disability, fatigue and pain as measured by a Multi-dimensional Health Assessment Questionnaire (MDHAQ). Clin Exp Rheumatol 2008; 26:S110.
  70. Akpolat T, Akkoyunlu M, Akpolat I, et al. Renal Behçet's disease: a cumulative analysis. Semin Arthritis Rheum 2002; 31:317.
  71. Melikoğlu M, Altiparmak MR, Fresko I, et al. A reappraisal of amyloidosis in Behçet's syndrome. Rheumatology (Oxford) 2001; 40:212.
  72. Göldeli O, Ural D, Komsuoğlu B, et al. Abnormal QT dispersion in Behçet's disease. Int J Cardiol 1997; 61:55.
  73. Huong DL, Wechsler B, Papo T, et al. Endomyocardial fibrosis in Behçet's disease. Ann Rheum Dis 1997; 56:205.
  74. Gürgün C, Ercan E, Ceyhan C, et al. Cardiovascular involvement in Behçet's disease. Jpn Heart J 2002; 43:389.
  75. Geri G, Wechsler B, Thi Huong du L, et al. Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature. Medicine (Baltimore) 2012; 91:25.
  76. Emmungil H, Yaşar Bilge NŞ, Küçükşahin O, et al. A rare but serious manifestation of Behçet's disease: intracardiac thrombus in 22 patients. Clin Exp Rheumatol 2014; 32:S87.
  77. Cheon JH, Kim WH. An update on the diagnosis, treatment, and prognosis of intestinal Behçet's disease. Curr Opin Rheumatol 2015; 27:24.
  78. Jung YS, Cheon JH, Park SJ, et al. Clinical course of intestinal Behcet's disease during the first five years. Dig Dis Sci 2013; 58:496.
  79. Seyahi E, Karaaslan H, Ugurlu S, Yazici H. Fever in Behçet's syndrome. Clin Exp Rheumatol 2013; 31:64.
  80. Erdoğru T, Koçak T, Serdaroğlu P, et al. Evaluation and therapeutic approaches of voiding and erectile dysfunction in neurological Behçet's syndrome. J Urol 1999; 162:147.
  81. Choung YH, Cho MJ, Park K, et al. Audio-vestibular disturbance in patients with Behçet's disease. Laryngoscope 2006; 116:1987.
  82. Kemal O, Anadolu Y, Boyvat A, Tatarağası A. Behçet disease as a cause of hearing loss: A prospective, placebo-controlled study of 29 patients. Ear Nose Throat J 2013; 92:112.
  83. Tada Y, Koarada S, Haruta Y, et al. The association of Behçet's disease with myelodysplastic syndrome in Japan: a review of the literature. Clin Exp Rheumatol 2006; 24:S115.
  84. Arimura K, Arima N, Matsushita K, et al. High incidence of morphological myelodysplasia and apoptotic bone marrow cells in Behçet's disease. J Clin Immunol 2007; 27:145.
  85. Lee SS, Yoon HJ, Chang HK, Park KS. Fibromyalgia in Behçet's disease is associated with anxiety and depression, and not with disease activity. Clin Exp Rheumatol 2005; 23:S15.
  86. Melikoglu M, Melikoglu MA. The prevalence of fibromyalgia in patients with Behçet's disease and its relation with disease activity. Rheumatol Int 2013; 33:1219.
  87. Jadaon J, Shushan A, Ezra Y, et al. Behçet's disease and pregnancy. Acta Obstet Gynecol Scand 2005; 84:939.
  88. Uzun S, Alpsoy E, Durdu M, Akman A. The clinical course of Behçet's disease in pregnancy: a retrospective analysis and review of the literature. J Dermatol 2003; 30:499.
  89. Noel N, Wechsler B, Nizard J, et al. Behçet's disease and pregnancy. Arthritis Rheum 2013; 65:2450.
  90. Iskender C, Yasar O, Kaymak O, et al. Behçet's disease and pregnancy: a retrospective analysis of course of disease and pregnancy outcome. J Obstet Gynaecol Res 2014; 40:1598.
  91. Alpsoy E. Behçet's disease: treatment of mucocutaneous lesions. Clin Exp Rheumatol 2005; 23:532.
  92. Yazici H, Tüzün Y, Pazarli H, et al. 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.
  93. Ferraz MB, Walter SD, Heymann R, Atra E. Sensitivity and specificity of different diagnostic criteria for Behçet's disease according to the latent class approach. Br J Rheumatol 1995; 34:932.
  94. Smith EL, Shmerling RH. The American College of Rheumatology criteria for the classification of systemic lupus erythematosus: strengths, weaknesses, and opportunities for improvement. Lupus 1999; 8:586.
  95. Dinc A, Bayir A, Simsek I, et al. The proportional Venn diagram of Behçet's disease-related manifestations among young adult men in Turkey. Clin Exp Rheumatol 2005; 23:S86.
  96. O'Neill TW, Rigby AS, Silman AJ, Barnes C. Validation of the International Study Group criteria for Behçet's disease. Br J Rheumatol 1994; 33:115.
  97. Tunç R, Uluhan A, Melikoğlu M, et al. A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome. Clin Exp Rheumatol 2001; 19:S45.
  98. Davatchi F, Schirmer M, Zouboulis C, et al, on behalf of the International Team for the Revision of the International Study Group Criteria for Bechet's disease. Evaluation and Revision of the International Study Group Criteria for Behcet's disease. Proceedings of the American College of Rheumatology Meeting; November 2007; Boston, MA. Abstract 1233.
  99. Davatchi F. Diagnosis/Classification Criteria for Behcet's Disease. Patholog Res Int 2012; 2012:607921.
  100. Barnes CG, Yazici H. Behçet's syndrome. Rheumatology (Oxford) 1999; 38:1171.
  101. Ambrose NL, Haskard DO. Differential diagnosis and management of Behçet syndrome. Nat Rev Rheumatol 2013; 9:79.