Advertisement

Behçet’s Disease

  • Erkan Alpsoy
Chapter

Abstract

Behçet’s disease (BD) is a chronic, relapsing inflammatory multisystem disease of unknown aetiology with the clinical features of mucocutaneous lesions and ocular, vascular, articular, neurologic, gastrointestinal, urogenital, pulmonary, and cardiac involvement [1, 2]. Mucocutaneous lesions are in the clinical presentation and can be considered the hallmarks of the disease. Their knowledge and recognition will permit earlier diagnosis and introduction of treatment with beneficial prognostic results for those affected. In most patients, the diagnosis can be made clinically on the basis of the mucocutaneous symptoms [2]. Since there is no pathognomonic test, multinational data have been recently revised, and International Criteria for Behçet’s Disease (ICBD) have been proposed by an international team as a guide for BD diagnosis and classification [3].

References

  1. 1.
    Behçet H. Uber rezidivierende aphthöse, durch ein Virus verursachte Geschwure, am Mund, am Auge, und an den Genitalien. Dermatol Wochenschr. 1937;105:1152–7.Google Scholar
  2. 2.
    Alpsoy E. Behçet’s disease: a comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions. J Dermatol. 2016;43:620–32.CrossRefPubMedGoogle Scholar
  3. 3.
    International Team for the Revision of the International Criteria for Behçet’s Disease (ITR-ICBD). The international criteria for Behçet’s disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014;28:338–47.CrossRefGoogle Scholar
  4. 4.
    Alpsoy E, Donmez L, Onder M, et al. Clinical features and natural course of Behcet’s disease in 661 cases: a multicentre study. Br J Dermatol. 2007;157:901–6.CrossRefPubMedGoogle Scholar
  5. 5.
    Azizlerli G, Kose AA, Sarica R, et al. Prevalence of Behcet’s disease in Istanbul, Turkey. Int J Dermatol. 2003;42:803–6.CrossRefPubMedGoogle Scholar
  6. 6.
    Assaad-Khalil SH, Kamel FA, Ismail EA. Starting a national registry for patients with Behçet’s disease in North West Nile Delta region in Egypt. In: Hamza M, editor. Behçet’s disease. Tunis (Tunisia): Pub Adhoua; 1979. p. 173–6.Google Scholar
  7. 7.
    Meda JR, Seni J, Mpondo B, Peck RN, Jaka H, Kilonzo SB. Behcet’s disease presenting with recurrent ocular, oral, and scrotal inflammatory lesions in a young Tanzanian man: a case report. Clin Case Rep. 2014;2:133–6.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM. Epidemiology and clinical characteristics of Behçet’s disease in the US: a population-based study. Arthritis Rheum. 2009;61(5):600–4.Google Scholar
  9. 9.
    Mahr A, Belarbi L, Wechsler B, et al. Population-based prevalence study of Behçet’s disease differences by ethnic origin and low variation by age at immigration. Arthritis Rheum. 2008;58:3951–9.CrossRefPubMedGoogle Scholar
  10. 10.
    Ekinci NS, Alpsoy E, Karakas AA, Yilmaz SB, Yegin O. IL-17A has an important role in the acute attacks of Behçet’s disease. J Invest Dermatol. 2010;130:2136–8.CrossRefPubMedGoogle Scholar
  11. 11.
    de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A. HLA-B51/B5 and the risk of Behcet's disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum. 2009;61:1287–96.CrossRefPubMedGoogle Scholar
  12. 12.
    Hughes T, Coit P, Adler A, et al. Identification of multiple independent susceptibility loci in the HLA region in Behçet’s disease. Nat Genet. 2013;45:319–24.CrossRefPubMedGoogle Scholar
  13. 13.
    Fei Y, Webb R, Cobb BL, Direskeneli H, Saruhan-Direskeneli G, Sawalha AH. Identification of novel genetic susceptibility loci for Behcet’s disease using a genome-wide association study. Arthritis Res Ther. 2009;11:R66.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Hou S, Yang Z, Du L, et al. Identification of a susceptibility locus in STAT4 for Behcet’s disease in Han Chinese in a genome-wide association study. Arthritis Rheum. 2012;64:4104–13.CrossRefPubMedGoogle Scholar
  15. 15.
    Kirino Y, Bertsias G, Ishigatsubo Y, et al. Genome-wide association analysis identifies new susceptibility loci for Behcet’s disease and epistasis between HLA-B-51 and ERAP1. Nat Genet. 2013;45:202–7.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Lee YJ, Horie Y, Wallace GR, et al. Genome-wide association study identifies GIMAP as a novel susceptibility locus for Behcet’s disease. Ann Rheum Dis. 2013;72:1510–6.CrossRefPubMedGoogle Scholar
  17. 17.
    Mizuki N, Meguro A, Ota M, et al. Genome-wide association studies identify IL23R-IL12RB2 and IL10 as Behcet’s disease susceptibility loci. Nat Genet. 2010;42:703–6.CrossRefPubMedGoogle Scholar
  18. 18.
    Remmers EF, Cosan F, Kirino Y, et al. Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behcet’s disease. Nat Genet. 2010;42:698–702.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Oh S, Han EC, Lee JH, Bang DT. Comparison of the clinical features of recurrent aphthous stomatitis and Behçet’s disease. Clin Exp Dermatol. 2009;34:e208–12.CrossRefPubMedGoogle Scholar
  20. 20.
    Alpsoy E, Zouboulis CC, Ehrlich CE. Mucocutaneous lesions of Behçet’s disease. Yonsei Med J. 2007;48:573–85.CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Kim B, LeBoit PE. Histopathologic features of erythema nodosum-like lesions in Behcet disease: a comparison with erythema nodosum focusing on the role of vasculitis. Am J Dermatopathol. 2000;22:379–90.CrossRefPubMedGoogle Scholar
  22. 22.
    Tunc R, Saip S, Siva A, Yazici H. Cerebral venous thrombosis is associated with major vessel disease in Behçet's syndrome. Ann Rheum Dis. 2004;63:1693–4.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Ugurlu N, Bozkurt S, Bacanli A, Akman-Karakas A, Uzun S, Alpsoy E. The natural course and factors affecting severity of Behçet’s disease: a single-center cohort of 368 patients. Rheumatol Int. 2015;35:2103–7.CrossRefPubMedGoogle Scholar
  24. 24.
    Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behcet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003;82:60–76.CrossRefGoogle Scholar
  25. 25.
    Ideguchi H, Suda A, Takeno M, Ueda A, Ohno S, Ishigatsubo Y. Behçet disease: evolution of clinical manifestations. Medicine (Baltimore). 2011;90:125–32.CrossRefGoogle Scholar
  26. 26.
    Bonitsis NG, Luong Nguyen LB, LaValley MP, et al. Gender-specific differences in Adamantiades-Behçet’s disease manifestations: an analysis of the German registry and meta-analysis of data from the literature. Rheumatology (Oxford). 2015;54:121–33.CrossRefGoogle Scholar
  27. 27.
    Yazici H, Tuzun Y, Pazarli H, et al. Influence of age of onset and patient’s sex on the prevalans and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis. 1984;43:783–9.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Dermatology and VenereologyAkdeniz University School of MedicineAntalyaTurkey

Personalised recommendations