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Behçet Syndrome pp 7–20Cite as

History and Diagnosis

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Abstract

Behçet syndrome, named after Hulusi Behçet, Professor of Dermatology in Istanbul who described the “triple symptom complex” in 1937, is a multisystem, immunologically medicated vasculopathy. The principal clinical features are orogenital aphthous ulceration, uveitis with or without hypopyon and retinal vasculitis, and skin lesions including erythema nodosum and acneiform pustules. Inflammatory arthritis, arterial and venous occlusion and arterial aneurysms, and gastrointestinal and neurological lesions also occur.

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References

  1. Behçet H. Uber rezidiverende, aphthose, durch ein virus verursachte gescgwure am mund, am auge und an den genitalen. Dermatol Wochenschr. 1937;105:1152–7.

    Google Scholar 

  2. Behçet H. Considerations sue les lesions aphtheuses de la bouche et des parties genitals, ainsi que sur les manifestations oculaires d’origine probablement virutique et observations concernant leur foyer d’infection. Bull Soc Fr Dermatol Syph. 1938;45:420–33.

    Google Scholar 

  3. Behçet H. Some observations on the clinical picture of the so-called triple symptom complex. Dermatologica. 1940;81:73–8.

    Article  Google Scholar 

  4. Saylan T. Commemorative lecture for Professor Dr. Hulusi Behçet. In: Dilşen N, Koniçe M, Övül C, editors. Behçet’s disease: proceedings of an international symposium on Behçet’s disease, Istanbul 29–30 September 1977. Excerpta Medica international congress series no. 467. Amsterdam-Oxford: Excerpta Medica; 1979. p. 1–5.

    Google Scholar 

  5. Saylan T. Life story of Dr. Hulusi Behçet. Yonsei Med J. 1997;38:327–32.

    Article  CAS  PubMed  Google Scholar 

  6. Dilsen N. History and development of Behçet’s disease. Rev Rhum Engl Ed. 1996;63:512–9.

    CAS  PubMed  Google Scholar 

  7. Lipschutz B. Ulcus vulvae acutum. In: Jadassohn J, editor. Handbuch der Haut- und Geschlechtskrankheiten, vol. 21. Berlin: Julius Springer; 1927. p. 392.

    Google Scholar 

  8. Fuchs H. Über chronische multiple Knoten-bildung am. Körper mit häufig rezidivierender eitriger Iritis und Skleritis. Dtsch Med Wochenschr. 1926;52:1503–5.

    Article  Google Scholar 

  9. Adams F. The genuine works of Hippocrates, translated from the Greek. London: The Sydenham Society; 1849. p. 403–4.

    Google Scholar 

  10. Feigenbaum A. Description of Behçet’s syndrome in the Hippocratic third book of endemic diseases. Br J Ophthalmol. 1956;40:355–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Gilbert W. Arch Augenheik. 1920;86:50–1.

    Google Scholar 

  12. Shigeta T. Recurrent iritis with hypopyon and its pathological findings. Acta Soc Ophthalmol Jpn. 1924;28:516–21.

    Google Scholar 

  13. Adamantiades B. Sur un cas d’iritis à hyopyon récidivante. Ann Ocul. 1931;168:271.

    Google Scholar 

  14. Adamantiades B, Lorando N. Sur le syndrome complexe de l’uveite recidivante ou soi-disant syndrome complexe de Behçet. Presse Med. 1949;57:501–3.

    CAS  PubMed  Google Scholar 

  15. Dascalopoulos N. Sur deux cas d’uveite recidivante. Ann Ocul. 1932;169:387–93.

    Google Scholar 

  16. Chauffard A, Brodin P, Wolf M. Stomatite et vulvite aphtheuses suivies de troubles dementiels passagers. Bull Mem Soc Med Hop Paris. 1923;47:841–4.

    Google Scholar 

  17. Neumann 1894: quoted by Wien MS, Perstein HO. Ulcus vulvae acutum associated with lesions of the mouth. JAMA. 1932;98:461–6.

    Article  Google Scholar 

  18. Planner H, Remenovsky F. Beitrage zur Kenntis der ulcerationen am ausseren weiblichen genitale. Arch Dermatol Syph Berl. 1922;111:162–88.

    Article  Google Scholar 

  19. Whitwell GPB. Recurrent buccal and vulval ulcers with associated embolic phenomena in skin and eyes. Br J Dermatol. 1934;46:414–9.

    Article  Google Scholar 

  20. Chajek T, Fainaru M. Behçet’s disease. Report of 41 cases and a review of the literature. Medicine (Baltimore). 1975;54:179–96.

    Article  CAS  Google Scholar 

  21. International Study Group for Behçet’s Disease. Criteria for diagnosis of Behçet’s disease. Lancet. 1990;335:1078–80.

    Google Scholar 

  22. International Study Group for Behçet’s Disease. Evaluation of diagnostic (‘classification’) criteria in Behçet’s disease – towards internationally agreed criteria. Br J Rheumatol. 1992;31:299–308.

    Article  Google Scholar 

  23. International Study Group for Behçet’s Disease. Evaluation of diagnostic (‘classification’) criteria in Behçet’s disease – towards internationally agreed criteria. In: O’Duffy JD, Kokmen E, editors. Behçet’s disease: basic and clinical aspects. New York, Basel, Hong Kong: Marcel Dekker, Inc.; 1991. p. 11–39.

    Google Scholar 

  24. Cooke BED. Oral ulceration in Behçet’s syndrome. In: Lehner T, Barnes CG, editors. Behçet’s syndrome: clinical and immunological features. London: Academic Press; 1979. p. 143–9.

    Google Scholar 

  25. Lehner T. Behçet’s syndrome and autoimmunity. Br Med J. 1967;1:465–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Sircus W, Church R, Kelleher J. Recurrent aphthous ulceration of the mouth. QJM. 1957;26:235–49.

    CAS  PubMed  Google Scholar 

  27. Koniçe M, Dilşen N, Aral O. The preaphthous phase of Behçet’s disease. In: Dilşen N, Koniçe M, Övül C, editors. Behçet’s disease: proceedings of an international symposium on Behçet’s disease, Istanbul 29–30 September 1977. Excerpta Medica international congress series no. 467. Amsterdam-Oxford: Excerpta Medica; 1979. p. 199–203.

    Google Scholar 

  28. Kim DK, Cahng SN, Bang D, et al. Clinical analysis of 40 cases of childhood-onset Behçet’s disease. Pediatr Dermatol. 1994;11:95–101.

    Article  CAS  PubMed  Google Scholar 

  29. Dunlop EMC. Genital and other manifestations of Behçet’s disease seen in venereological practice. In: Lehner T, Barnes CG, editors. Behçet’s syndrome: clinical and immunological features. London: Academic Press; 1979. p. 159–75.

    Google Scholar 

  30. Dinning WJ. An overview of ocular manifestations. In: Lehner T, Barnes CG, editors. Recent advances in Behçet’s disease. Royal Society of Medicine Services international congress and symposium series no. 103. London, New York: Royal Society of Medicine Services; 1986. p. 227–33.

    Google Scholar 

  31. Kansu T, Kadayifcilar S. Visual aspects of Behçet’s disease. Curr Neurol Neurosci Rep. 2005;5:382–8.

    Article  PubMed  Google Scholar 

  32. Kitaichi N, Miyazaki A, Iwata D, et al. Ocular features of Behçet’s disease: an international collaborative study. Br J Ophthalmol. 2007;91:1573–4.

    Article  Google Scholar 

  33. Kaçmaz RO, Kempen JH, Newcomb C, et al. Ocular inflammation in Behçet’s disease: incidence of ocular complications and of loss of visual acuity. Am J Ophthalmol. 2008;146:828–36.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Yazici H, Pazerli H, Barnes CG, et al. A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med. 1990;322:281–5.

    Article  CAS  PubMed  Google Scholar 

  35. Hamuryudan V, Ozyazgan Y, Fresko I, et al. Interferon-alfa combined with azathioprine for the uveitis of Behçet’s disease: an open study. Isr Med Assoc J. 2002;4:928–30.

    CAS  PubMed  Google Scholar 

  36. Krause L, Altenburg A, Pleyer U, et al. Longterm visual prognosis of patients with ocular Adamantiades-Behçet’s disease treated with interferon-alpha-2a. J Rheumatol. 2008;35:896–903.

    CAS  PubMed  Google Scholar 

  37. Nazzaro P. Cutaneous manifestations of Behçet’s disease; clinical and histopathological findings. In: Monacelli M, Nazzaro P, editors. Behçet’s disease. Basel, New York: S. Karger; 1966. p. 15–41.

    Google Scholar 

  38. Chun SI, Su WP, Lee S, et al. Erythema nodosum-like lesions in Behçet’s syndrome: a histopathologic study of 30 cases. J Cutan Pathol. 1989;16:259–65.

    Article  CAS  PubMed  Google Scholar 

  39. Lee ES, Bang D, Lee S. Dermatologic manifestations of Behçet’s disease. Yonsei Med J. 1997;38:380–9.

    Article  CAS  PubMed  Google Scholar 

  40. Alpsoy E, Zouboulis CC, Ehrlich GE. Mucocutaneous lesions of Behçet’s disease. Yonsei Med J. 2007;48:573–85.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Yazici H, Fresco I, Yurdakul S. Behçet’s syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol. 2007;3:148–55.

    Article  PubMed  Google Scholar 

  42. Kienbaum S, Zouboulis CC, Waibel M, et al. Chemotactic neutrophilic vasculitis: a new histological pattern of vasculitis found in mucocutaneous lesions of patients with Adamantiades-Behçet’s disease. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 337–41.

    Google Scholar 

  43. Kienbaum S, Zouboulis CC, Waibel M, et al. Papulopustular skin lesions in Adamantiades-Behçet’s disease show a similar histopathological pattern as the classical mucocutaneous manifestations. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 331–6.

    Google Scholar 

  44. Melikoglu M, Kural-Seyahi E, Tascilar K, et al. The unique features of vasculitis in Behçet’s syndrome. Clin Rev Allergy Immunol. 2008;35:40–6.

    Article  CAS  PubMed  Google Scholar 

  45. Blobner F. Zur rezidivierenden hypopyon-iritis. Z Augenheik. 1937;91:129–39.

    Google Scholar 

  46. Jensen T. Sur les ulcerations aphtheuses de la muqueues de la bouche et de la peau genitale combines avec les symptoms oculaires (Syndrome de Behçet). Acta Dermatol Venereol. 1941;22:64–79.

    Google Scholar 

  47. Yazici H, Tüzün Y, Pazarli H, et al. The combined use of HLA-B5 and the pathergy test as diagnostic markers of Behçet’s disease in Turkey. J Rheumatol. 1980;7:206–10.

    CAS  PubMed  Google Scholar 

  48. Davies PG, Fordham JN, Kirwan JR, et al. The pathergy test and Behcet’s syndrome in Britain. Ann Rheum Dis. 1984;43:70–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Yazici H, Chamberlain MA, Tüzün Y, et al. A comparative study of the pathergy reaction among Turkish and British patients with Behçet’s disease. Ann Rheum Dis. 1984;43:74–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  50. Yazici H, Tüzün Y, Tanman S, et al. Male patients with Behçet’s syndrome have stronger pathergy reactions. Clin Exp Rheumatol. 1985;3:137–41.

    CAS  PubMed  Google Scholar 

  51. Suzuki K, Mizuno N. Intracutaneous test with physiological saline in Behcet’s disease. In: Inaba G, editor. Behçet’s disease: pathogenetic mechanism and clinical future. Japanese Medical Research Foundation publication no. 18. Tokyo: University of Tokyo Press; 1982. p. 333–42.

    Google Scholar 

  52. Dilşen N, Koniçe M, Aral O, et al. Comparative study of the skin pathergy test with blunt and sharp needles in Behçet’s disease: confirmed specificity but decreased sensitivity with sharp needles. Ann Rheum Dis. 1993;52:823–5.

    Article  PubMed  PubMed Central  Google Scholar 

  53. Fresko I, Yazici H, Bayramiçli M, et al. Effect of surgical cleaning of the skin on the pathergy phenomenon in Behçet’s syndrome. Ann Rheum Dis. 1993;52:619–20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  54. Hatemi I, Hatemi G, Celik AF, et al. Frequency of pathergy phenomenon and other features of Behçet’s syndrome among patients with inflammatory bowel disease. Clin Exp Rheumatol. 2008;26:591–5.

    Google Scholar 

  55. Dilşen N, Koniçe M, Aral O, et al. Standardization and evaluation of the skin pathergy test in Behçet’s disease and controls. In: Lehner T, Barnes CG, editors. Recent advances in Behçet’s disease. Royal Society of Medicine Services international congress and symposium series no. 103. London, New York: Royal Society of Medicine Services; 1986. p. 169–72.

    Google Scholar 

  56. Aral O, Dilşen N, Koniçe M, et al. Positive skin pathergy reactivity as a genetic marker of Behçet’s disease. In: Lehner T, Barnes CG, editors. Recent advances in Behçet’s disease. Royal Society of Medicine Services international congress and symposium series no. 103. London, New York: Royal Society of Medicine Services; 1986. p. 173–5.

    Google Scholar 

  57. Dilşen N, Koniçe M, Aral O, et al. Important implications of skin pathergy test in Behçet’s disease. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 229–33.

    Google Scholar 

  58. Cakir N, Yazici H, Chamberlain MA, et al. Response to intradermal injection of monosodium urate crystals in Behçet’s syndrome. Ann Rheum Dis. 1991;50:634–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  59. Strachan RW, Wigzell FW. Polyarthritis in Behçet’s multiple symptom complex. Ann Rheum Dis. 1963;22:26–35.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  60. Oshima Y, Shimizu T, Yokohari R, et al. Clinical studies on Behçet’s syndrome. Ann Rheum Dis. 1963;22:36–45.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. Mason RM, Barnes CG. Behçet-syndrom mit arthritis. Schweiz Med Wochenschr. 1968;98:665–71.

    CAS  PubMed  Google Scholar 

  62. Mason RM, Barnes CG. Behçet’s syndrome with arthritis. Ann Rheum Dis. 1969;28:95–103.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  63. Nasr F. Les manifestations articulaires de la maladie de Behçet. Rev Rheumatol. 1969;36:81–3.

    CAS  Google Scholar 

  64. Bisson M, Amor B, Kahan A, et al. Les manifestations articulaires de l’aphthose (syndrome de Behçet). Sem Hôp Paris. 1971;47:2024–33.

    Google Scholar 

  65. Dilşen N, Koniçe M, Övül C. Arthritis patterns in Behçet’s disease. In: Dilşen N, Koniçe M, Övül C, editors. Behçet’s disease: proceedings of an international symposium on Behçet’s disease, Istanbul 29–30 September 1977. Excerpta Medica international congress series no. 467. Amsterdam-Oxford: Excerpta Medica; 1979. p. 145–53.

    Google Scholar 

  66. Yurdakul S, Yazici H, Tüzün Y, et al. The arthritis of Behçet’s disease: a prospective study. Ann Rheum Dis. 1983;42:505–15.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  67. Dawes PT, Raman D, Haslock I. Acute synovial rupture in Behçet’s syndrome. Ann Rheum Dis. 1983;42:591–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  68. Hashimoto T, Shimizu T. Immunohistopathological studies on arthritis in Behçet’s syndrome. Scand J Rheumatol. 1975;4(Suppl 8):36–8.

    Google Scholar 

  69. Vernon-Roberts B, Barnes CG, Revell PA. Synovial pathology in Behçet’s syndrome. Ann Rheum Dis. 1978;37:139–45.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  70. Ben-Dov I, Zimmerman J. Deforming arthritis of the hands in Behçet’s disease. J Rheumatol. 1982;9:617–8.

    CAS  PubMed  Google Scholar 

  71. Currey HLF, Elson RA, Mason M. Surgical treatment of manubrio-sternal pain in Behçet’s syndrome. J Bone Joint Surg. 1968;50B:836–40.

    Article  Google Scholar 

  72. Takeuchi A, Mori M, Hashimoto A. Radiographic abnormalities in patients with Behçet’s disease. Clin Exp Rheumatol. 1984;2:259–62.

    CAS  PubMed  Google Scholar 

  73. Jawad ASM, Goodwill CJ. Behçet’s disease with erosive arthritis. Ann Rheum Dis. 1986;45:961–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  74. Dilşen N, Koniçe M, Aral O. Why Behçet’s disease should be accepted as a seronegative arthritis. In: Lehner T, Barnes CG, editors. Recent Advances in Behçet’s disease. Royal Society of Medicine Services international congress and symposium series no. 103. London, New York: Royal Society of Medicine Services; 1986. p. 281–4.

    Google Scholar 

  75. Moll JMH, Haslock MD, Macrae IF, et al. Associations between ankylosing spondylitis, psoriatic arthritis, Reiter’s disease, the intestinal arthropathies, and Behçet’s syndrome. Medicine. 1974;53:343–64.

    Article  CAS  PubMed  Google Scholar 

  76. Yazici H, Turunc M, Özdoğan H, et al. Observer variation in grading sacroiliac radiographs might be a cause of ‘sacroiliitis’ reported in certain disease states. Ann Rheum Dis. 1987;46:139–45.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  77. Chamberlain MA, Robertson RJH. A controlled study of sacroiliitis in Behçet’s disease. Br J Rheumatol. 1993;32:693–8.

    Article  CAS  PubMed  Google Scholar 

  78. Scavo D, Cramarossa L. Discussion. In: Monacelli M, Nazzaro P, editors. Behçet’s disease. Basel, New York: S. Karger; 1966. p. 137–8.

    Google Scholar 

  79. Shimizu T. Clinicopathological studies on Behçet’s disease. In: Dilşen N, Koniçe M, Övül C, editors. Behçet’s disease: proceedings of an international symposium on Behçet’s disease, Istanbul 29–30 September 1977. Excerpta Medica international congress series no. 467. Amsterdam-Oxford: Excerpta Medica; 1979. p. 9–43.

    Google Scholar 

  80. Shimizu T. Vascular lesions of Behçet’s disease. Cardioangiology. 1977;1:124.

    Google Scholar 

  81. Shimizu T. Behçet’s disease: a systemic inflammatory disease. In: Vascular lesions of collagen diseases and related conditions. Tokyo: Tokyo University Press; 1977. p. 201–11.

    Google Scholar 

  82. Bayraktar Y, Balkanci F, Demirkazik F, et al. Type of vessel involvement in patients with Behçet’s disease. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 331–6.

    Google Scholar 

  83. Hamza M. Angio Behcet. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 523–6.

    Google Scholar 

  84. Dong Y, Liu J. Vasculo-Behcet’s disease. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 527–30.

    Google Scholar 

  85. El-Ramahi KM, Al-Dalaan A, Al-Balaa S, et al. Vascular involvement in Behçet disease. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 531–6.

    Google Scholar 

  86. Demirkazik FB, Balkanci F, Çekirge S, et al. Vascular involvement in Behçet’s disease. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 537–40.

    Google Scholar 

  87. Yazici H, Yurdakul S, Hamuryudan V, et al. The vasculitides: Behçet’s syndrome. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, editors. Rheumatology. 3rd ed. Barcelona: Elsevier; 2003. p. 1665–9.

    Google Scholar 

  88. Lehner T. Immunopathogenesis of Behçet’s disease. In: Bang D, Lee E-S, Lee S, editors. Behcet’s disease: proceedings of the international conference on Behçet’s disease, held in Seoul, Korea, May 27–29, 2000. Seoul: Design Mecca Publishing Co.; 2000. p. 3–18.

    Google Scholar 

  89. Haskard DO, Chambers JC. Kooner JS Impaired vascular endothelial function in Behçet’s syndrome can be restored by vitamin C. In: Bang D, Lee E-S, Lee S, editors. Behcet’s disease: proceedings of the international conference on Behçet’s disease, held in Seoul, Korea, May 27–29, 2000. Seoul: Design Mecca Publishing Co.; 2000. p. 229.

    Google Scholar 

  90. Chambers JC, Haskard DO, Kooner JS. Vascular endothelial function and oxidative stress mechanisms in patients with Behçet’s syndrome. J Am Coll Cardiol. 2001;37:517–20.

    Article  CAS  PubMed  Google Scholar 

  91. Kaiser EDE, Ozyazgan Y, Rao NA. Immunohistopathology of Behçet’s disease. In: Zierhut M, Ohno S, editors. Immunology of Behcet’s disease. Lisse: Swets & Zeitlinger B.V; 2003. p. 47–55.

    Google Scholar 

  92. Dilşen N, Koniçe M, Gazioğlu K, et al. Pleuropulmonary manifestations in Behçet’s disease. In: Dilşen N, Koniçe M, Övül C, editors. Behçet’s disease: proceedings of an international symposium on Behçet’s disease, Istanbul 29–30 September 1977. Excerpta Medica international congress series no. 467. Amsterdam-Oxford: Excerpta Medica; 1979. p. 163–73.

    Google Scholar 

  93. Okamoto S, Kimura T, Masugi Y, et al. A case report of Behcet’s syndrome which showed a massive haemoptysis. J Jpn Soc Intern Med. 1969;58:1268.

    Google Scholar 

  94. Petty TL, Scoggin CH, Good JT. Recurrent pneumonia in Behçet’s syndrome. JAMA. 1977;238:2529–30.

    Article  CAS  PubMed  Google Scholar 

  95. Efthimiou J, Spiro SG. Pulmonary involvement in Behçet’s syndrome. In: Lehner T, Barnes CG, editors. Recent advances in Behçet’s disease. Royal Society of Medicine Services international congress and symposium series no. 103. London, New York: Royal Society of Medicine Services; 1986. p. 261–6.

    Google Scholar 

  96. Erkan F, Gül A, Tasali E. Pulmonary manifestations of Behçet’s disease. Thorax. 2001;56:572–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  97. Hamuryudan V, Er T, Seyahi E, et al. Pulmonary artery aneurysms in Behçet syndrome. Am J Med. 2004;117:867–70.

    Article  PubMed  Google Scholar 

  98. Yazici H, Esen F. Mortality in Behçet’s syndrome. Clin Exp Rheumatol. 2008;26(Suppl 51):S138–40.

    CAS  PubMed  Google Scholar 

  99. Berlin C. Behçet’s syndrome with involvement of central nervous system: report of a case of necropsy. Arch Dermatol Syph. 1944;49:227.

    Article  Google Scholar 

  100. Evans AD, Pallis CA, Spillane JD. Involvement of the nervous system in Behçet’s syndrome; report of three cases and isolation of virus. Lancet. 1957;273:349–53.

    Article  CAS  PubMed  Google Scholar 

  101. Rubinstein LJ, Urich H. Meningoencephalitis of Behçet’s disease: case report with pathological findings. Brain. 1963;86:151–60.

    Article  CAS  PubMed  Google Scholar 

  102. Pallis CA. Behçet’s disease and the nervous system. Trans St Johns Hosp Dermatol Soc. 1966;52:201–6.

    CAS  PubMed  Google Scholar 

  103. Pallis CA, Fudge BJ. The neurological complications of Behcet’s syndrome. AMA Arch Neurol Psyhchiatr. 1956;75:1–14.

    Article  CAS  Google Scholar 

  104. Al-Araji A, Sharquie K, Al-Rawi Z. Prevalence and patterns of neurological involvement in Behcet’s disease: a prospective study from Iraq. J Neurol Neurosurg Psychiatry. 2003;74:608–13.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  105. Kidd D. The prevalence of headache in Behçet’s syndrome. Rheumatology (Oxford). 2006;45:621–3.

    Article  CAS  Google Scholar 

  106. Fukuda Y, Hayashi H, Kuwara N. Pathological studies on Neuro-Behçet’s disease. In: Inaba G, editor. Behçet’s disease: pathogenetic mechanism and clinical future. Japanese Medical Research Foundation publication no. 18. Tokyo: University of Tokyo Press; 1982. p. 137–43.

    Google Scholar 

  107. Al-Araji A, Kidd DP. Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol. 2009;8:192–204.

    Article  PubMed  Google Scholar 

  108. Khosravi F, Samangooei S. The long term clinical outcome of central nervous system involvement in Behçet’s disease. In: Bang D, Lee E-S, Lee S, editors. Behcet’s disease: proceedings of the international conference on Behçet’s disease, held in Seoul, Korea, May 27–29, 2000. Seoul: Design Mecca Publishing Co.; 2000. p. 302–6.

    Google Scholar 

  109. Emmi L, Salvati G, Li Gobbi F, et al. A SPECT study protocol for the evaluation of cerebral blood flow alterations in Behçet’s disease. In: Bang D, Lee E-S, Lee S, editors. Behcet’s disease: proceedings of the international conference on Behçet’s disease, held in Seoul, Korea, May 27–29, 2000. Seoul: Design Mecca Publishing Co.; 2000. p. 307–11.

    Google Scholar 

  110. Boe J, Dalgaard JB, Scott D. Mucocutaneous-ocular syndrome with intestinal involvement; a clinical and pathological study of four fatal cases. Am J Med. 1958;25:857–67.

    Article  CAS  PubMed  Google Scholar 

  111. O’Duffy JD, Carney JA, Deodhar S. Behçet’s disease. Report of 10 cases, 3 with new manifestations. Ann Intern Med. 1971;75:561–70.

    Article  PubMed  Google Scholar 

  112. Fukuda Y, Watanabe I. Pathological studies on intestinal Behçet’s (Entero-Behçet’s) disease. In: Dilşen N, Koniçe M, Övül C, editors. Behçet’s disease: proceedings of an international symposium on Behçet’s disease, Istanbul 29–30 September 1977. Excerpta Medica international congress series no. 467. Amsterdam-Oxford: Excerpta Medica; 1979. p. 90–103.

    Google Scholar 

  113. Sladen GE, Lehner T. Gastrointestinal disorders in Behcet’s syndrome and a comparison with recurrent oral ulcers. In: Lehner T, Barnes CG, editors. Behçet’s syndrome: clinical and immunological features. London: Academic Press; 1979. p. 151–8.

    Google Scholar 

  114. Hamza M. Pharyngeal stenosis in Behçet’s disease. Clin Exp Rheumatol. 1988;6:139–40.

    CAS  PubMed  Google Scholar 

  115. Houman MH, Ben Ghorbel I, Lamloum M, et al. Esophageal involvement in Behcet’s disease. Yonsei Med J. 2002;43:457–60.

    Article  PubMed  Google Scholar 

  116. Taylor CB, Low N, Raj S, et al. Behçet’s syndrome progressing to gastrointestinal perforation in a West African male. Br J Rheumatol. 1997;36:498–501.

    Article  CAS  PubMed  Google Scholar 

  117. Dowling CM, Hill ADK, Malone C, et al. Colonic perforation in Behçet’s syndrome. World J Gastroenterol. 2008;14:6578–80.

    Article  PubMed  PubMed Central  Google Scholar 

  118. Ebert EC. Gastrointestinal manifestations of Behçet’s disease. Dig Dis Sci. 2009;54(2):201–7.

    Article  PubMed  Google Scholar 

  119. Sezer FN. The isolation of a virus as the cause of Behcet’s diseases. Am J Ophthalmol. 1956;36:301–15.

    Article  Google Scholar 

  120. Lehner T, Barnes CG. Criteria for diagnosis and classification of Behçet’s syndrome. In: Lehner T, Barnes CG, editors. Behçet’s syndrome: clinical and immunological features. London: Academic Press; 1979. p. 1–9.

    Google Scholar 

  121. Dilşen N, Koniçe M, Övül C, et al. A preliminary family study on Behçet’s disease in Turkey. In: Inaba G, editor. Behçet’s disease: pathogenetic mechanism and clinical future. Japanese Medical Research Foundation publication no. 18. Tokyo: University of Tokyo Press; 1982. p. 103–11.

    Google Scholar 

  122. Ohno S, Aoki K, Sugiura S, et al. HL-A5 and Behçet’s disease. Lancet. 1973;2:1383–4.

    Article  CAS  PubMed  Google Scholar 

  123. Yurdakul S, Günaydin I, Tüzün Y, et al. The prevalence of Behçet’s syndrome in a rural area of northern Turkey. J Rheumatol. 1988;15:820–2.

    CAS  PubMed  Google Scholar 

  124. O’Duffy JD. Summary of international symposium on Behçet’s disease. J Rheumatol. 1978;5:229–33.

    PubMed  Google Scholar 

  125. Chamberlain MA. Behcet’s syndrome in 32 patients in Yorkshire. Ann Rheum Dis. 1977;36:491–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  126. Jankowski J, Crombie I, Jankowski R. Behçet’s syndrome in Scotland. Postgrad Med J. 1992;68:566–70.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  127. O’Neill TW, Silman AJ, Rigby AS, et al. Sex and regional differences in clinical manifestations of Behçet’s disease. Br J Rheumatol. 1993;32(Suppl 1):85.

    Google Scholar 

  128. 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;43:783–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  129. Hamza M. Juvenile Behçet’s disease. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 377–80.

    Google Scholar 

  130. Shafaie N, Shahram F, Davatcghi F, et al. Behçet’s disease in children. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 381–3.

    Google Scholar 

  131. Koné-Paut I, Bernard J-L. Behçet’s disease in children: a French nationwide survey. In: Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993. p. 385–9.

    Google Scholar 

  132. Zouboulis CC, Kötter I, Djawari D, et al. Epidemiological features of Adamantiades-Behçet disease in Germany and in Europe. Yonsei Med J. 1997;38:411–22.

    Article  CAS  PubMed  Google Scholar 

  133. Behcet’s Disease Research Committee of Japan. Behcet’s disease guide to diagnosis of Behcet’s disease. Jpn J Ophthalmol. 1974;18:291–4.

    Google Scholar 

  134. Mizushima Y. Recent research into Behcet’s disease in Japan. Int J Tissue React. 1988;10:59–65.

    CAS  PubMed  Google Scholar 

  135. Suzuki Kurokawa M, Suzuki N. Behcet’s disease. Clin Exp Med. 2004;4:10–20.

    Article  CAS  PubMed  Google Scholar 

  136. Davatchi F, Sadeghi Abdollahi B, Shahram F, Nadji A, Chams-Davatchi C, Shams H, et al. Validation of the International Criteria for Behçet’s disease (ICBD) in Iran. Int J Rheum Dis. 2010;13:55–60.

    Article  PubMed  Google Scholar 

  137. Blake T, et al. Birmingham Behçet’s service: classification of disease and application of the 2014 International Criteria for Behçet’s Disease (ICBD) to a UK cohort. BMC Musculoskelet Disord. 2017;18:101.

    Article  PubMed  PubMed Central  Google Scholar 

  138. Yazici H, Seyahi E, Hatemi G, Yazici Y. Behçet syndrome: a contemporary view. Nat Rev Rheumatol. 2018;14:107–19.

    Article  CAS  PubMed  Google Scholar 

  139. Marchionini A, Müller E. The dermatological view of morbus Hulusi Behçet. In: Monacelli M, Nazzaro P, editors. Behçet’s disease. Basel, New York: S. Karger; 1966. p. 6–14.

    Google Scholar 

  140. Monacelli M, Nazzaro P, editors. Behçet’s disease. Basel, New York: S. Karger; 1966.

    Google Scholar 

  141. Dilşen N, Koniçe M, Övül C, editors. Behçet’s disease: proceedings of an international symposium on Behçet’s disease, Istanbul 29–30 September 1977. Excerpta Medica international congress series no. 467. Amsterdam-Oxford: Excerpta Medica; 1979.

    Google Scholar 

  142. Inaba G, editor. Behçet’s disease: pathogenetic mechanism and clinical future. Japanese Medical Research Foundation publication no. 18. Tokyo: University of Tokyo Press; 1982.

    Google Scholar 

  143. Lehner T, Barnes CG, editors. Recent advances in Behçet’s disease. Royal Society of Medicine Services international congress and symposium series no. 103. London, New York: Royal Society of Medicine Services; 1986.

    Google Scholar 

  144. O’Duffy JD, Kokmen E, editors. Behçet’s disease: basic and clinical aspects. New York, Basel, Hong Kong: Marcel Dekker Inc.; 1991.

    Google Scholar 

  145. Wechsler B, Godeau P, editors. Behçet’s disease. International congress series 1037. Amsterdam, London, New York, Tokyo: Excerpta Medica; 1993.

    Google Scholar 

  146. Hamza M, editor. Behcet’s disease: proceeding of the seventh international conference on Behçet’s disease held at Tunis, October 10–11, 1996. Tunisia: Pub Adhoua. ISBN 9973-17-850-5.

    Google Scholar 

  147. Bang D, Lee E-S, Lee S, editors. Behçet’s disease: proceedings of the 8th international conference on Behçet’s disease, held in Reggio Emilia, Italy, October 7–9, 1998. Seoul: Design Mecca Publishing Co.. ISBN 89-951655-1-0.

    Google Scholar 

  148. Bang D, Lee E-S, Lee S, editors. Behçet’s disease: proceedings of the international conference on Behçet’s disease, held in Seoul, Korea, May 27–29, 2000. Seoul: Design Mecca Publishing Co.. ISBN 89-951655-1-0.

    Google Scholar 

  149. Zouboulis CC, editor. Adamantiades-Behçet’s disease. Advances in experimental medicine and biology, vol. 528. New York: Kluwer Academic/Plenum Publishers; 2003.

    Google Scholar 

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Author information

Authors and Affiliations

  1. (Formerly of the) Department of Rheumatology, The Royal London Hospital, London, UK

    Colin G. Barnes

  2. (Formerly of the) Queen Mary College, University of London, London, UK

    Colin G. Barnes

  3. Little Hoopern, Chagford, UK

    Colin G. Barnes

  4. European League Against Rheumatism, Zürich, Switzerland

    Colin G. Barnes

  5. School of Medicine, New York University, New York, NY, USA

    Yusuf Yazici

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  1. Colin G. Barnes
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Editors and Affiliations

  1. School of Medicine, New York University, New York, NY, USA

    Yusuf Yazici

  2. Department of Internal Medicine, Istanbul University, Istanbul, Turkey

    Gulen Hatemi

  3. Department of Rheumatology, Istanbul University, Istanbul, Turkey

    Emire Seyahi

  4. Department of Medicine, Academic Hospital, Istanbul, Turkey

    Hasan Yazici

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Barnes, C.G., Yazici, Y. (2020). History and Diagnosis. In: Yazici, Y., Hatemi, G., Seyahi, E., Yazici, H. (eds) Behçet Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-24131-5_2

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