Behcet Disease

Reference work entry

In 1937, Behçet first described three patients with oral and genital ulceration and hypopyon. It is most common in the Middle and Far East with a prevalence of 7–8 per 100,000 people, whereas in the United States the prevalence is estimated at 4 per 1,000,000 people.

Synonyms and Related Disorders

Behcet syndrome

Genetics/Basic Defect

  1. 1.

    A complex multisystem inflammatory disease of unknown cause

  2. 2.
    Considered a relapsing and remitting vasculitis of the small- to medium-sized vessels with following protean manifestations:
    1. a.

      Aphthous stomatitis

    2. b.

      Genital ulceration

    3. c.


    4. d.


    5. e.

      Gastrointestinal lesions

    6. f.

      Cutaneous lesions

    7. g.

      Central nervous system lesions

    8. h.

      Cardiac lesions

    9. i.

      Vessel lesions

  3. 3.
    Immunopathogenic aspects of Behcet disease (Direskeneli 2001; Krause and Weinberger 2008)
    1. a.

      Neutrophil activation

    2. b.

      Cellular and humoral immunity

    3. c.
      Antigenic stimuli
      1. i.

        Herpes simplex virus

      2. ii.

        Streptococci and superantigens

      3. iii.

        Heat shock...


Anterior Uveitis Genital Ulcer Behcet Disease Posterior Uveitis Retinal Vasculitis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. Alpsoy, E., & Akman, A. (2009). Behçet’s disease: An algorithmic approach to its treatment. Archives of Dermatological Research, 301, 693–702.PubMedCrossRefGoogle Scholar
  2. Alpsoy, E., Zouboulis, C. C., & Ehrlich, G. E. (2007). Mucocutaneous lesions of Behçet’s disease. Yonsei Medical Journal, 48, 573–585.PubMedCrossRefGoogle Scholar
  3. Bashour, M. (2010). Behcet disease. eMedicine from WebMD. Updated February 16, 2010. Available at:
  4. Behçet, H. (1937). Über rezidivierende, aphthöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatology Wochenschrift, 105, 1152–1157.Google Scholar
  5. Benezra, D., & Cohen, E. (1986). Treatment and visual prognosis in Behcet’s disease. British Journal of Ophthalmology, 70, 589–592.PubMedCrossRefGoogle Scholar
  6. Brik, R., Shamali, H., & Bergman, R. (2001). Successful thalidomide treatment of severe infantile Behçet disease. Pediatric Dermatology, 18, 143.PubMedCrossRefGoogle Scholar
  7. Ceylan, N., Bayraktaroglu, S., Erturk, S. M., et al. (2010). Pulmonary and vascular manifestations of Behcet disease: Imaging findings. American Journal of Roentgenology, 194, 158–164.CrossRefGoogle Scholar
  8. Chae, E. J., Do, K.-H., Seo, J. B., et al. (2008). Radiologic and clinical findings of Behçet disease: Comprehensive review of multisystemic involvement. Radiographics, 28, 1–55.CrossRefGoogle Scholar
  9. Chajek, T., & Fainaru, M. (1975). Behcet’s disease: Report of 41 cases and review of the literature. Medicine, 54, 179–196.PubMedCrossRefGoogle Scholar
  10. Chen, K. R., Kawara, Y., Miyakawa, S., et al. (1997). Cutaneous vasculitis in Behçets disease. A clinical and histopathological study of 20 patients. Journal of the American Academy of Dermatology, 36, 689–696.PubMedCrossRefGoogle Scholar
  11. Direskeneli, H. (2001). Behcet’s disease: Infectious aetiology, new autoantigens, and HLA-B51. Annals of the Rheumatic Diseases, 60, 996–1002.PubMedCrossRefGoogle Scholar
  12. Diri, E., & Espinoza, L. R. (2006). Neuro-Behçet’s syndrome: Differential diagnosis and management. Current Rheumatology Reports, 8, 317–322.PubMedCrossRefGoogle Scholar
  13. Ebert, E. C. (2009). Gastrointestinal manifestations of Behçet’s disease. Digestive Diseases and Sciences, 54, 201–207.PubMedCrossRefGoogle Scholar
  14. Ecker, R. D., Galiani, D., Carney, M., et al. (2000). Behcets disease in an African American man. The American Journal of Emergency Medicine, 18, 118–119.PubMedCrossRefGoogle Scholar
  15. Englin, R. P., Lehner, T., & Subak-Sharpe, J. H. (1982). Detection of RNA complementary to herpes simplex virus in mononuclear cells from patients with Behcets syndrome and recurrent oral ulcers. Lancet, 2, 1356–1361.CrossRefGoogle Scholar
  16. Evereklioglu, C., Inalöz, H. S., Kirtak, N., et al. (2002). Serum leptin concentration is increased in patients with Behçet’s syndrome and is correlated with disease activity. British Journal of Dermatology, 147, 331.PubMedCrossRefGoogle Scholar
  17. George, R. K., Chan, C. C., Whitcup, S. M., et al. (1997). Ocular immunopathology of Behcet’s disease. Survey of Ophthalmology, 42, 157–162.PubMedCrossRefGoogle Scholar
  18. Georgiou, S., Monastirli, A., Pasmatzi, E., et al. (1998). Efficacy and safety of systemic recombinant interferon-alpha in Behcet’s disease. Journal of International Medicine, 243, 367–372.Google Scholar
  19. Ghate, J. V., & Jorizzo, J. L. (1999). Behçets disease and complex aphthosis. Journal of the American Academy of Dermatology, 40, 1–18.PubMedCrossRefGoogle Scholar
  20. Hamuryudan, V., Mat, C., Saip, S., et al. (1998). Thalidomide in the treatment of the mucocutaneous lesions of the Behcet syndrome. A randomized, double-blind, placebo-controlled trial. Annals of Internal Medicine, 128, 443–450.PubMedGoogle Scholar
  21. International Study Group for Behcet’s Disease. (1990). Criteria for diagnosis of Behcet’s Disease. Lancet, 335, 1078–1080.Google Scholar
  22. Jorizzo, J. L., Abernethy, J. L., White, W. L., et al. (1995). Mucocutaneous criteria for the diagnosis of Behçets disease: An analysis of clinicopathologic data from multiple international centers. Journal of the American Academy of Dermatology, 32, 968–976.PubMedCrossRefGoogle Scholar
  23. Krause, I., & Weinberger, A. (2008). Behçet’s disease. Current Opinion in Rheumatology, 20, 82–87.PubMedCrossRefGoogle Scholar
  24. Krespi, Y., Akman-Demir, G., Poyraz, M., et al. (2001). Cerebral vasculitis and ischaemic stroke in Behçet’s disease: Report of one case and review of the literature. European Journal of Neurology, 8, 719.PubMedCrossRefGoogle Scholar
  25. Kurokawa, M. S., & Suzuki, N. (2004). Behcet’s disease. Clinical and Experimental Medicine, 3, 10–20.CrossRefGoogle Scholar
  26. Lang, B., Laxer, R., Thorin, P., et al. (1990). Pediatric onset of Behçet’s syndrome with myositis: Case report and literature review illustrating unusual features. Arthritis and Rheumatism, 33, 418–424.PubMedCrossRefGoogle Scholar
  27. Mangelsdorf, H. C., White, W. L., & Jorizzo, J. L. (1996). Behçet’s disease: Report of twenty-five patients from United States with prominent mucocutaneous involvement. Journal of the American Academy of Dermatology, 34, 745–750.PubMedCrossRefGoogle Scholar
  28. Mogulkoc, N., Burgess, M. I., & Bishop, P. W. (2000). Intracardiac thrombus in Behcet’s disease: A systemic review. Chest, 118, 479–487.PubMedCrossRefGoogle Scholar
  29. O’Duffy, J. D., & Goldstein, N. P. (1976). Neurologic involvement in seven patients with Behcet’s disease. The American Journal of Medicine, 61, 17–18.Google Scholar
  30. Önder, M., & Gürer, M. A. (2001). The multiple faces of Behçet’s disease and its aetiological factors. Journal of the European Academy of Dermatology and Venereology, 15, 126.PubMedCrossRefGoogle Scholar
  31. Piuetti Pezzi, P., Gasparri, V., De Liso, P., et al. (1985). Prognosis in Behcet’s Disease. Annals of Ophthalmology, 17, 20–25.Google Scholar
  32. Posadas. A. C., & Lisse, J. R. (2009). Behcet disease. eMedicine from WebMD. Updated June 22, 2009. Available at:
  33. Rabinovich, C. E., & Wagner-Weiner, L. (2009). Behcet syndrome. eMedicine from WebMD. Updated August 24, 2009. Available at:
  34. Rizzi, R., Bruno, S., & Dammacco, R. (1997). Behçet’s disease: An immune-mediated vasculitis involving vessels of all sizes. International Journal of Clinical and Laboratory Research, 27, 225–232.PubMedCrossRefGoogle Scholar
  35. Sakane, T., Takeno, M., Suzuki, N., et al. (1999). Behçet’s disease. The New England Journal of Medicine, 341, 1284–1291.PubMedCrossRefGoogle Scholar
  36. Serdaroglu, P. (1998). Behçet’s disease and the nervous system. Journal of Neurology, 245, 197–205.PubMedCrossRefGoogle Scholar
  37. Shek, L. P. C., Lee, Y. S., & Lehman, T. J. A. (1999). Thalidomide responsiveness in an infant with Behçet’s syndrome. Pediatrics, 103, 1295–1297.PubMedCrossRefGoogle Scholar
  38. Stratigos, A., Laskaris, G., & Stratigos, J. (1992). Behçet’s disease. Seminars in Neurology, 12, 346–357.PubMedCrossRefGoogle Scholar
  39. Verity, D. H., Marr, J. E., Ohno, S., et al. (1999). Behçet’s disease, the silk road and HLA-B51: Historical and geographical perspectives. Tissue Antigens, 54, 213–220.PubMedCrossRefGoogle Scholar
  40. Yazici, H. (1997). The place of Behcet’s syndrome among the autoimmune diseases. International Reviews of Immunology, 14, 1–10.PubMedCrossRefGoogle Scholar
  41. Yousefi, M., Ferringer, T., Lee, S., et al. (2009). Behcet disease. eMedicine from WebMD. Updated June 19, 2009. Available at:
  42. Zen-nyoji, M., Okamura, S. I., Kyoko Harada, K., et al. (2000). Intestinal Behcet’s disease associated with generalized myositis. Gastrointestinal Endoscopy, 51, 359–361.PubMedCrossRefGoogle Scholar

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