Behçet Disease

Living reference work entry

Abstract

In 1937, Behçet (1937) 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 USA the prevalence is estimated at 4 per 1,000,000 people.

Keywords

Corticosteroid Interferon Cyclosporine Meningitis Perforation 

References

  1. Alnaimat, F. A. (2015). Behcet disease. eMedicine from WebMD. Updated 23 Dec 2015. Available at: http://emedicine.medscape.com/article/329099-overview
  2. Alpsoy, E., & Akman, A. (2009). Behçet’s disease: An algorithmic approach to its treatment. Archives of Dermatological Research, 301, 693–702.CrossRefPubMedGoogle Scholar
  3. Alpsoy, E., Zouboulis, C. C., & Ehrlich, G. E. (2007). Mucocutaneous lesions of Behçet’s disease. Yonsei Medical Journal, 48, 573–585.CrossRefPubMedPubMedCentralGoogle Scholar
  4. Bashour, M. (2014). Behcet disease. eMedicine from WebMD. Updated 3 Mar 2014. Available at: http://emedicine.medscape.com/article/1229174-overview
  5. Behçet, H. (1937). Über rezidivierende, aphthöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatologische Wochenschrift, 105, 1152–1157.Google Scholar
  6. Benezra, D., & Cohen, E. (1986). Treatment and visual prognosis in Behcet’s disease. British Journal of Ophthalmology, 70, 589–592.CrossRefPubMedPubMedCentralGoogle Scholar
  7. Brik, R., Shamali, H., & Bergman, R. (2001). Successful thalidomide treatment of severe infantile Behçet disease. Pediatric Dermatology, 18, 143.CrossRefPubMedGoogle Scholar
  8. 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
  9. 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
  10. Chajek, T., & Fainaru, M. (1975). Behcet’s disease: Report of 41 cases and review of the literature. Medicine, 54, 179–196.CrossRefPubMedGoogle Scholar
  11. 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.CrossRefPubMedGoogle Scholar
  12. Dalvi, S. R., Yildirim, R., & Yazici, Y. (2012). Behçet’s syndrome. Drugs, 72, 2223–2241.CrossRefPubMedGoogle Scholar
  13. Direskeneli, H. (2001). Behcet’s disease: Infectious aetiology, new autoantigens, and HLA-B51. Annals of the Rheumatic Diseases, 60, 996–1002.CrossRefPubMedPubMedCentralGoogle Scholar
  14. Diri, E., & Espinoza, L. R. (2006). Neuro-Behçet’s syndrome: Differential diagnosis and management. Current Rheumatology Reports, 8, 317–322.CrossRefPubMedGoogle Scholar
  15. Ebert, E. C. (2009). Gastrointestinal manifestations of Behçet’s disease. Digestive Diseases and Sciences, 54, 201–207.CrossRefPubMedGoogle 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.CrossRefPubMedGoogle Scholar
  17. Georgiou, S., Monastirli, A., Pasmatzi, E., et al. (1998). Efficacy and safety of systemic recombinant interferon-alpha in Behcet’s disease. Journal of Internal Medicine, 243, 367–372.CrossRefPubMedGoogle Scholar
  18. Ghate, J. V., & Jorizzo, J. L. (1999). Behçets disease and complex aphthosis. Journal of the American Academy of Dermatology, 40, 1–18.CrossRefPubMedGoogle Scholar
  19. Gul, A., Tugal-Tutkun, I., Dinarello, C., et al. (2012). Interleukin-1beta-regulating antibody XOMA 052 (gevokizumab) in the treatment of acute exacerbations of resistant uveitis of Behcet’s disease: An open-label pilot study. Annals of the Rheumatic Diseases, 71, 563–566.CrossRefPubMedGoogle 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.CrossRefPubMedGoogle Scholar
  21. International Study Group for Behcet’s Disease. (1990). Criteria for diagnosis of Behcet’s Disease. Lancet, 335, 1078–1080.Google Scholar
  22. Iskender, C., Yasar, O., Kaymak, O., et al. (2014). Behçet disease and pregnancy: A retrospective analysis of course of disease and pregnancy outcome. Journal of Obstetrics and Gynaecology Research, 40, 1598–1602.CrossRefPubMedGoogle Scholar
  23. Krause, I., & Weinberger, A. (2008). Behçet’s disease. Current Opinion in Rheumatology, 20, 82–87.CrossRefPubMedGoogle 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.CrossRefPubMedGoogle 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.CrossRefPubMedGoogle 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.CrossRefPubMedGoogle Scholar
  28. Mogulkoc, N., Burgess, M. I., & Bishop, P. W. (2000). Intracardiac thrombus in Behcet’s disease: A systemic review. Chest, 118, 479–487.CrossRefPubMedGoogle Scholar
  29. Noel, N., Wechsler, B., Nizard, J., et al. (2013). Behçet’s disease and pregnancy. Arthritis and Rheumatism, 65, 2450–2456.CrossRefPubMedGoogle Scholar
  30. 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
  31. Ognenovski, M., Renauer, P., & Gensterblum, E. (2015). Whole exome sequencing identifies rare protein-coding variants in Behçet’s disease. Arthritis and Rheumatology, December 14, 1–27 [Epub ahead of print].Google Scholar
  32. 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.CrossRefPubMedGoogle Scholar
  33. Pivetti Pezzi, P., Gasparri, V., De Liso, P., et al. (1985). Prognosis in Behcet’s disease. Annals of Ophthalmology, 17, 20–25.PubMedGoogle Scholar
  34. Rabinovich, C. E. (2014). Behcet syndrome. eMedicine from WebMD. Updated 1 May 2014. Available at: http://emedicine.medscape.com/article/1006358-overview
  35. 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.CrossRefPubMedGoogle Scholar
  36. Sadreddini, S., Noshad, H., Molaeefard, M., et al. (2008). Treatment of retinal vasculitis in Behcet’s disease with rituximab. Modern Rheumatology, 18, 306–308.CrossRefPubMedGoogle Scholar
  37. Sakane, T., Takeno, M., Suzuki, N., et al. (1999). Behçet’s disease. The New England Journal of Medicine, 341, 1284–1291.CrossRefPubMedGoogle Scholar
  38. Saleh, Z., & Arayssi, T. (2014). Update on the therapy of Behçet’s disease. Therapeutic Advances in Chronic Disease, 5, 112–134.CrossRefPubMedPubMedCentralGoogle Scholar
  39. Serdaroglu, P. (1998). Behçet’s disease and the nervous system. Journal of Neurology, 245, 197–205.CrossRefPubMedGoogle Scholar
  40. Seyahi, E., & Yazici, H. (2015). Behçet’s syndrome: pulmonary vascular disease. Current Opinion in Rheumatology, 27, 18–23.CrossRefPubMedGoogle Scholar
  41. Seyahi, E., Cakmak, O. S., Tutar, B., et al. (2015). Clinical and ultrasonographic evaluation of lower-extremity vein thrombosis in Behcet syndrome: An observational study. Medicine, 94, e1899.CrossRefPubMedGoogle Scholar
  42. 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.CrossRefPubMedGoogle Scholar
  43. Skef, W., Hamilton, M. J., & Arayssi, T. (2015). Gastrointestinal Behçet’s disease: A review. World Journal of Gastroenterology, 21, 3801–3812.CrossRefPubMedPubMedCentralGoogle Scholar
  44. Ugurlu, S., Ucar, D., Seyahi, E., et al. (2012). Canakinumab in a patient with juvenile Behcet’s syndrome with refractory eye disease. Annals of the Rheumatic Diseases, 71, 1589–1591.CrossRefPubMedGoogle Scholar
  45. 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.CrossRefPubMedGoogle Scholar
  46. Yazici, H. (1997). The place of Behcet’s syndrome among the autoimmune diseases. International Reviews of Immunology, 14, 1–10.CrossRefPubMedGoogle Scholar
  47. 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.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  1. 1.Medical GeneticsShriners Hospitals for ChildrenShreveportUSA
  2. 2.Perinatal and Clinical Genetics, Department of PediatricsLSU Health Sciences CenterShreveportUSA

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