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Behcet Disease

  • Reference work entry
Atlas of Genetic Diagnosis and Counseling

  • 247 Accesses

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.

      Uveitis

    4. d.

      Synovitis

    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

        ...

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References

  • Alpsoy, E., & Akman, A. (2009). Behçet’s disease: An algorithmic approach to its treatment. Archives of Dermatological Research, 301, 693–702.

    Article  PubMed  Google Scholar 

  • Alpsoy, E., Zouboulis, C. C., & Ehrlich, G. E. (2007). Mucocutaneous lesions of Behçet’s disease. Yonsei Medical Journal, 48, 573–585.

    Article  PubMed  CAS  Google Scholar 

  • Bashour, M. (2010). Behcet disease. eMedicine from WebMD. Updated February 16, 2010. Available at: http://emedicine.medscape.com/article/1229174-overview

  • Behçet, H. (1937). Ãœber rezidivierende, aphthöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatology Wochenschrift, 105, 1152–1157.

    Google Scholar 

  • Benezra, D., & Cohen, E. (1986). Treatment and visual prognosis in Behcet’s disease. British Journal of Ophthalmology, 70, 589–592.

    Article  PubMed  CAS  Google Scholar 

  • Brik, R., Shamali, H., & Bergman, R. (2001). Successful thalidomide treatment of severe infantile Behçet disease. Pediatric Dermatology, 18, 143.

    Article  PubMed  CAS  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  Google Scholar 

  • Chajek, T., & Fainaru, M. (1975). Behcet’s disease: Report of 41 cases and review of the literature. Medicine, 54, 179–196.

    Article  PubMed  CAS  Google Scholar 

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • Direskeneli, H. (2001). Behcet’s disease: Infectious aetiology, new autoantigens, and HLA-B51. Annals of the Rheumatic Diseases, 60, 996–1002.

    Article  PubMed  CAS  Google Scholar 

  • Diri, E., & Espinoza, L. R. (2006). Neuro-Behçet’s syndrome: Differential diagnosis and management. Current Rheumatology Reports, 8, 317–322.

    Article  PubMed  Google Scholar 

  • Ebert, E. C. (2009). Gastrointestinal manifestations of Behçet’s disease. Digestive Diseases and Sciences, 54, 201–207.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • George, R. K., Chan, C. C., Whitcup, S. M., et al. (1997). Ocular immunopathology of Behcet’s disease. Survey of Ophthalmology, 42, 157–162.

    Article  PubMed  CAS  Google Scholar 

  • 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.

    CAS  Google Scholar 

  • Ghate, J. V., & Jorizzo, J. L. (1999). Behçets disease and complex aphthosis. Journal of the American Academy of Dermatology, 40, 1–18.

    Article  PubMed  CAS  Google Scholar 

  • 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.

    PubMed  CAS  Google Scholar 

  • International Study Group for Behcet’s Disease. (1990). Criteria for diagnosis of Behcet’s Disease. Lancet, 335, 1078–1080.

    Google Scholar 

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • Krause, I., & Weinberger, A. (2008). Behçet’s disease. Current Opinion in Rheumatology, 20, 82–87.

    Article  PubMed  CAS  Google Scholar 

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • Kurokawa, M. S., & Suzuki, N. (2004). Behcet’s disease. Clinical and Experimental Medicine, 3, 10–20.

    Article  Google Scholar 

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • Mogulkoc, N., Burgess, M. I., & Bishop, P. W. (2000). Intracardiac thrombus in Behcet’s disease: A systemic review. Chest, 118, 479–487.

    Article  PubMed  CAS  Google Scholar 

  • 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 

  • 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.

    Article  PubMed  Google Scholar 

  • Piuetti Pezzi, P., Gasparri, V., De Liso, P., et al. (1985). Prognosis in Behcet’s Disease. Annals of Ophthalmology, 17, 20–25.

    Google Scholar 

  • Posadas. A. C., & Lisse, J. R. (2009). Behcet disease. eMedicine from WebMD. Updated June 22, 2009. Available at: http://emedicine.medscape.com/article/329099-overview

  • Rabinovich, C. E., & Wagner-Weiner, L. (2009). Behcet syndrome. eMedicine from WebMD. Updated August 24, 2009. Available at: http://emedicine.medscape.com/article/1006358-overview

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • Sakane, T., Takeno, M., Suzuki, N., et al. (1999). Behçet’s disease. The New England Journal of Medicine, 341, 1284–1291.

    Article  PubMed  CAS  Google Scholar 

  • Serdaroglu, P. (1998). Behçet’s disease and the nervous system. Journal of Neurology, 245, 197–205.

    Article  PubMed  CAS  Google Scholar 

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • Stratigos, A., Laskaris, G., & Stratigos, J. (1992). Behçet’s disease. Seminars in Neurology, 12, 346–357.

    Article  PubMed  CAS  Google Scholar 

  • 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.

    Article  PubMed  CAS  Google Scholar 

  • Yazici, H. (1997). The place of Behcet’s syndrome among the autoimmune diseases. International Reviews of Immunology, 14, 1–10.

    Article  PubMed  CAS  Google Scholar 

  • Yousefi, M., Ferringer, T., Lee, S., et al. (2009). Behcet disease. eMedicine from WebMD. Updated June 19, 2009. Available at: http://emedicine.medscape.com/article/1122381-overview

  • 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.

    Article  PubMed  CAS  Google Scholar 

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Editor information

Editors and Affiliations

  1. Perinatal and Clinical Genetics, Department of Pediatrics, LSU Health Sciences Center, 1501 Kings Highway, Shreveport, LA, 71130, USA

    Harold Chen

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(2012). Behcet Disease. In: Chen, H. (eds) Atlas of Genetic Diagnosis and Counseling. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-1037-9_23

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