Medscape is available in 5 Language Editions – Choose your Edition here.


Dermatologic Aspects of Behcet Disease

  • Author: Amira M Elbendary, MBBCh, MSc; Chief Editor: William D James, MD  more...
Updated: Jan 12, 2016


Behçet disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who described the triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis ,[1] but it is likely that Behçet disease was already described years earlier by Hippocrates, who described a disease with aphthous ulcers and defluxion around genital area, associated with warty ophthalmic conditions.[2]

Behçet disease is a complex, multisystemic disease that includes involvement of the mucocutaneous, ocular, cardiovascular, renal, gastrointestinal, pulmonary, urologic, and central nervous systems, as well as the joints, blood vessels, and lungs.



The cause of Behçet disease is not known; however, immunogenetics, immune regulation, vascular abnormalities (including arterial and venous abnormalities, affecting small, medium, and large vessels), or bacterial and viral infection may have a role in its development.




United States

Behçet disease is not common in the United States, with a prevalence of 5 cases per 100,000 persons.


A wide geographic variation is noted in the frequency of Behçet disease, with high prevalence in Mediterranean area compared with the United States and Northern Europe. Studies have shown that immigrants from areas with high prevalence rate of Behçet disease remain at high risk of developing Behçet disease.[3] Behçet disease is most prevalent (and more virulent) in the Mediterranean region, Middle East, and Far East, with an estimated prevalence of 1 case per 10,000 persons.

The prevalence reported in different countries is as follows[3] :

  • Turkey: 20-420 cases per 100,000 population
  • Iran: 16-68 cases per 100,000 population
  • Israel: 15-120 cases per 100,000 population
  • Iraq: 17 cases per 100,000 population
  • Egypt: 7.6 cases per 100,000 population
  • Spain: 7.5 cases per 100,000 population
  • Japan: 7-13.5 cases per 100,000 population
  • Martinique: 7 cases per 100,000 population
  • France: 7.1 cases per 100,000 population
  • Italy: 3.8-16 cases per 100,000 population
  • Northern Europe: 0.5-5 cases per 100,000 population


Men are affected more often, and with more severe disease, than women in some Mediterranean areas. In Iran, for example, the male-to-female ratio was 24:1 among 1712 patients. In Turkey, the ratio was 16:1 among 427 patients.


Onset can occur at any age, but is it most common during the third decade of life.[4] Behçet disease rarely occurs in individuals older than 50 years or during childhood.[5] Genetic anticipation was reported in Behçet disease, in which the onset of the disease becomes earlier with successive generations.[6]



Chronic morbidity is typical; the leading cause is ophthalmic involvement, which can result in blindness. The effects of the disease may be cumulative, especially with neurologic, vascular, and ocular involvement.

The mortality rate is low, but death can occur from neurologic involvement, vascular disease, bowel perforation, cardiopulmonary disease, or as a complication of immunosuppressive therapy.[7]

A recent study has shown that sub-Saharan African patients with Behçet disease had three times higher mortality compared with North African and European patients, with the 15-year mortality rate reaching 20%, compared with mortality rates of about 5-10% reported in other countries.[8]

Clinical expression of the disease also shows geographic variation. Sub-Saharan African patients show higher CNS and cardiovascular involvement compared with Behçet disease patients in North Africa and Europe.

Gastrointestinal manifestations and neurological involvement was found to occur at higher rates in American patients compared with Turkish patients.[8]

Higher rates of folliculitis were found in patients of Iraqi/Iranian origin, and ocular complications were found to be at higher incidence in patients of Arabic origin in a study done on 66 Jewish patients.[8]



The clinical course of Behçet disease is variable, even in the early stages, making determinations of the long-term prognosis difficult.

Men appear to have a poorer prognosis.

The disease usually runs a protracted course, with attacks generally lasting for several weeks and recurring more frequently early in the disease.

Mucocutaneous and arthritic involvement usually occur early.

Chronic morbidity is usual; the leading cause is ophthalmic involvement, which can result in blindness. The effects of the disease may be cumulative, especially for neurologic, vascular, and ocular involvement.

Mortality is low, but patients may die from neurologic involvement, vascular disease, bowel perforation, cardiopulmonary disease, or as a complication of immunosuppressive therapy.

The risk of cancer was evaluated in a large cohort study including 1,314 patients with Behçet disease. Non-Hodgkin lymphoma, hematologic malignancies, and female breast cancer were the most frequent malignancies observed. The cancer risk was found to be highest within the first year of follow up.[9]


Patient Education

For patient education resources, see the Teeth and Mouth Center, as well as Canker Sores.

Contributor Information and Disclosures

Amira M Elbendary, MBBCh, MSc Visiting Research Fellow, Ackerman Academy of Dermatopathology; Teaching Assistant, Department of Dermatology, Kasr Alainy University Hospital, Cairo University, Egypt

Amira M Elbendary, MBBCh, MSc is a member of the following medical societies: Medical Dermatology Society, Bloom’s Syndrome Association, Egyptian Medical Syndicate, International Dermoscopy Society

Disclosure: Nothing to disclose.


Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

David P Fivenson, MD Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan

David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Michigan State Medical Society, Society for Investigative Dermatology, Photomedicine Society, Wound Healing Society, Michigan Dermatological Society, Medical Dermatology Society

Disclosure: Nothing to disclose.


Dongsik Bang, MD, PhD Vice Director, Severance Hospital; Professor, Department of Dermatology, Yonsei University College of Medicine, Korea

Disclosure: Nothing to disclose.

Tammie Ferringer, MD Dermatopathology Section Head, Dermatopathology Fellowship Director, Departments of Dermatology and Pathology, Geisinger Medical Center

Tammie Ferringer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, and International Society of Dermatopathology

Disclosure: Nothing to disclose.

Eun-So Lee, MD, PhD Chairman, Professor of Dermatology, Department of Dermatology, Ajou University School of Medicine, Korea

Disclosure: Nothing to disclose.

Sungnack Lee, MD Vice President of Medical Affairs, Professor, Department of Dermatology, Ajou University School of Medicine, Korea

Sungnack Lee, MD is a member of the following medical societies: American Dermatological Association

Disclosure: Nothing to disclose.

Marjan Yousefi, MD Department of Dermatology, Geisinger Medical Center

Marjan Yousefi, MD is a member of the following medical societies: American Academy of Dermatology and Phi Beta Kappa

Disclosure: Nothing to disclose.

  1. Behcet H. Uber rezidivierende, aphthose, durchein Virus verursachte Geschwure am Mund, am Auge und anden Genitalien. Dermatol Wochenschr. 1937. 36:1152-7.

  2. Feigenbaum A. Description of Behçet's syndrome in the Hippocratic third book of endemic diseases. Br J Ophthalmol. 1956 Jun. 40(6):355-7. [Medline]. [Full Text].

  3. Mahr A, Maldini C. [Epidemiology of Behçet's disease]. Rev Med Interne. 2014 Feb. 35(2):81-9. [Medline].

  4. Kim DK, Chang SN, Bang D, Lee ES, Lee S. Clinical analysis of 40 cases of childhood-onset Behcet's disease. Pediatr Dermatol. 1994 Jun. 11(2):95-101. [Medline].

  5. Mat C, Yurdakul S, Sevim A, Özyazgan Y, Tüzün Y. Behçet's syndrome: facts and controversies. Clin Dermatol. 2013 Jul-Aug. 31(4):352-61. [Medline].

  6. Gülbay B, Acican T, Erçen Diken Ö, Pinar Önen Z. Familial Behçet's disease of adult age: a report of 4 cases from a Behçet family. Intern Med. 2012. 51(12):1609-11. [Medline].

  7. 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 Dec. 25(6):857-67. [Medline].

  8. Savey L, Resche-Rigon M, Wechsler B, Comarmond C, Piette JC, Cacoub P, et al. Ethnicity and association with disease manifestations and mortality in Behçet's disease. Orphanet J Rare Dis. 2014 Mar 27. 9:42. [Medline]. [Full Text].

  9. Wang LH, Wang WM, Hsu SM, Lin SH, Shieh CC. Risk of Overall and Site-specific Cancers in Behçet Disease: A Nationwide Population-based Study in Taiwan. J Rheumatol. 2015 May. 42 (5):879-84. [Medline].

  10. Mizushima Y. [Revised diagnostic criteria for Behcet's disease in 1987]. Ryumachi. 1988 Feb. 28(1):66-70. [Medline].

  11. International Study Group for Behcet's Disease. Criteria for diagnosis of Behcet's disease. Lancet. 1990 May 5. 335(8697):1078-80. [Medline].

  12. 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 Mar. 28(3):338-47. [Medline].

  13. Yurdakul S, Yazici H. Behçet's syndrome. Best Pract Res Clin Rheumatol. 2008 Oct. 22(5):793-809. [Medline].

  14. Sharquie KE, Al-Araji A, Hatem A. Oral pathergy test in Behçet's disease. Br J Dermatol. 2002 Jan. 146(1):168-9. [Medline].

  15. Ideguchi H, Suda A, Takeno M, Ueda A, Ohno S, Ishigatsubo Y. Behçet disease: evolution of clinical manifestations. Medicine (Baltimore). 2011 Mar. 90(2):125-32. [Medline].

  16. Mat MC, Goksugur N, Engin B, Yurdakul S, Yazici H. The frequency of scarring after genital ulcers in Behçet's syndrome: a prospective study. Int J Dermatol. 2006 May. 45(5):554-6. [Medline].

  17. Lee ES, Bang D, Lee S. Dermatologic manifestation of Behçet's disease. Yonsei Med J. 1997 Dec. 38(6):380-9. [Medline].

  18. Kim B, LeBoit PE. Histopathologic features of erythema nodosum--like lesions in Behçet disease: a comparison with erythema nodosum focusing on the role of vasculitis. Am J Dermatopathol. 2000 Oct. 22(5):379-90. [Medline].

  19. Diri E, Mat C, Hamuryudan V, Yurdakul S, Hizli N, Yazici H. Papulopustular skin lesions are seen more frequently in patients with Behçet's syndrome who have arthritis: a controlled and masked study. Ann Rheum Dis. 2001 Nov. 60(11):1074-6. [Medline]. [Full Text].

  20. Azizlerli G, Ozarmagan G, Ovül C, Sarica R, Mustafa SO. A new kind of skin lesion in Behçet's disease: extragenital ulcerations. Acta Derm Venereol. 1992 Aug. 72(4):286. [Medline].

  21. Ozarmagan G, Saylan T, Azizlerli G, Ovül C, Aksungur VL. Re-evaluation of the pathergy test in Behçet's disease. Acta Derm Venereol. 1991. 71(1):75-6. [Medline].

  22. Fresko I, Yazici H, Bayramiçli M, Yurdakul S, Mat C. Effect of surgical cleaning of the skin on the pathergy phenomenon in Behçet's syndrome. Ann Rheum Dis. 1993 Aug. 52(8):619-20. [Medline]. [Full Text].

  23. Aytekin S, Yuksel EP, Aydin F, Senturk N, Ozden MG, Canturk T, et al. Nailfold capillaroscopy in Behçet disease, performed using videodermoscopy. Clin Exp Dermatol. 2014 Jun. 39(4):443-7. [Medline].

  24. Vaiopoulos G, Pangratis N, Samarkos M, Hatzinicolaou P, Mavropoulos S, Tzonou A, et al. Nailfold capillary abnormalities in Behçet's disease. J Rheumatol. 1995 Jun. 22(6):1108-11. [Medline].

  25. Movasat A, Shahram F, Carreira PE, Nadji A, Akhlaghi M, Naderi N, et al. Nailfold capillaroscopy in Behçet's disease, analysis of 128 patients. Clin Rheumatol. 2009 May. 28(5):603-5. [Medline].

  26. Mezalek ZT, Harmouche H, Attar NE, Serraj K, Aouni M, Adnaoui M, et al. Kaposi's sarcoma in association with Behcet's disease: case report and literature review. Semin Arthritis Rheum. 2007 Apr. 36(5):328-31. [Medline].

  27. Sibley C, Yazici Y, Tascilar K, Khan N, Bata Y, Yazici H, et al. Behçet syndrome manifestations and activity in the United States versus Turkey -- a cross-sectional cohort comparison. J Rheumatol. 2014 Jul. 41(7):1379-84. [Medline].

  28. Tugal-Tutkun I, Urgancioglu M. Childhood-onset uveitis in Behcet disease:a descriptive study of 36 cases. Am J Ophthalmol. 2003 Dec. 136(6):1114-9. [Medline].

  29. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, Huseyin Altunbas H, Urgancioglu M. Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol. 2004 Sep. 138(3):373-80. [Medline].

  30. Aridogan BC, Yildirim M, Baysal V, Inaloz HS, Baz K, Kaya S. Serum Levels of IL-4, IL-10, IL-12, IL-13 and IFN-gamma in Behcet's disease. J Dermatol. 2003 Aug. 30(8):602-7. [Medline].

  31. Saadoun D, Wechsler B, Resche-Rigon M, et al. Cerebral venous thrombosis in Behcet's disease. Arthritis Rheum. 2009 Apr 15. 61(4):518-26. [Medline].

  32. Desbois AC, Wechsler B, Cluzel P, Helft G, Boutin D, Piette JC, et al. [Cardiovascular involvement in Behçet's disease]. Rev Med Interne. 2014 Feb. 35(2):103-11. [Medline].

  33. Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003 Jan. 82(1):60-76. [Medline].

  34. Ando M, Kosakai Y, Okita Y, Nakano K, Kitamura S. Surgical treatment of Behçet's disease involving aortic regurgitation. Ann Thorac Surg. 1999 Dec. 68(6):2136-40. [Medline].

  35. Seyahi E, Melikoglu M, Yazici H. Clinical features and diagnosis of Behcet's syndrome. Int J Adv Rheumatol. 2007. 5:8.

  36. Seyahi E, Cakmak OS, Tutar B, Arslan C, Dikici AS, Sut N, et al. Clinical and Ultrasonographic Evaluation of Lower-extremity Vein Thrombosis in Behcet Syndrome: An Observational Study. Medicine (Baltimore). 2015 Nov. 94 (44):e1899. [Medline].

  37. Ng MY, Boutet A, Carette S, de Perrot M, Cusimano RJ, Nguyen ET. Behcet Disease Presenting With Cardiac and Pulmonary Masses. Can J Cardiol. 2015 Sep. 31 (9):1204.e5-7. [Medline].

  38. Lee YP, Cho CH, Chi HS, et al. Intestinal Behcet's disease. J Korean Surg Soc. 1988. 35:310-5.

  39. Choi IJ, Kim JS, Cha SD, Jung HC, Park JG, Song IS, et al. Long-term clinical course and prognostic factors in intestinal Behçet's disease. Dis Colon Rectum. 2000 May. 43(5):692-700. [Medline].

  40. Kim NH, Yang IH, Kim SM, Bang D. Behcet's arthritis. J Korean Orthop Surg. 1993. 28:1890-7.

  41. Dalvi SR, Yildirim R, Yazici Y. Behcet's Syndrome. Drugs. 2012 Dec 3. 72(17):2223-41. [Medline].

  42. Alonso S, Riveros-Frutos A, Martínez-Morillo M, Grau-Ferrer L, Carrato C, Olivé A. Pseudotumoral Behçet's disease. Reumatol Clin. 2015 Jun 12. [Medline].

  43. Lee J, Cho S, Kim do Y, Zheng Z, Park H, Bang D. Carpal Tunnel Syndrome in Behçet's Disease. Yonsei Med J. 2015 Jul. 56 (4):1015-20. [Medline].

  44. van Ham C, Schrijvers D, De Picker L, Vandendriessche F, Sabbe B. Neuropsychiatric features in Behçet's disease: A case report. Clin Neurol Neurosurg. 2014 Dec. 127:13-4. [Medline].

  45. Yazmalar L, Batmaz I, Sariyildiz MA, Yildiz M, Uçmak D, Türkçü F, et al. Sleep quality in patients with Behçet's disease. Int J Rheum Dis. 2014 Sep 8. [Medline].

  46. Kanda N, Watanabe S. Regulatory roles of sex hormones in cutaneous biology and immunology. J Dermatol Sci. 2005 Apr. 38(1):1-7. [Medline].

  47. Iskender C, Yasar O, Kaymak O, Yaman ST, Uygur D, Danisman N. Behçet's disease and pregnancy: a retrospective analysis of course of disease and pregnancy outcome. J Obstet Gynaecol Res. 2014 Jun. 40(6):1598-602. [Medline].

  48. Saleh Z, Arayssi T. Update on the therapy of Behçet disease. Ther Adv Chronic Dis. 2014 May. 5(3):112-34. [Medline]. [Full Text].

  49. Noel N, Wechsler B, Nizard J, Costedoat-Chalumeau N, Boutin du LT, Dommergues M, et al. Behçet's disease and pregnancy. Arthritis Rheum. 2013 Sep. 65(9):2450-6. [Medline].

  50. Jadaon J, Shushan A, Ezra Y, Sela HY, Ozcan C, Rojansky N. Behçet's disease and pregnancy. Acta Obstet Gynecol Scand. 2005 Oct. 84(10):939-44. [Medline].

  51. Atmaca L, Boyvat A, Yalçindag FN, Atmaca-Sonmez P, Gurler A. Behçet disease in children. Ocul Immunol Inflamm. 2011 Apr. 19(2):103-7. [Medline].

  52. Chang YS, Yang YH, Chiang BL. Neonatal Behçet's disease without maternal history. Clin Rheumatol. 2011 Dec. 30(12):1641-5. [Medline].

  53. Metreau-Vastel J, Mikaeloff Y, Tardieu M, Koné-Paut I, Tran TA. Neurological involvement in paediatric Behçet's disease. Neuropediatrics. 2010 Oct. 41(5):228-34. [Medline].

  54. Koné-Paut I, Yurdakul S, Bahabri SA, Shafae N, Ozen S, Ozdogan H, et al. Clinical features of Behçet's disease in children: an international collaborative study of 86 cases. J Pediatr. 1998 Apr. 132(4):721-5. [Medline].

  55. Sungur GK, Hazirolan D, Yalvac I, Ozer PA, Yuksel D, Vural ET, et al. Clinical and demographic evaluation of Behçet disease among different paediatric age groups. Br J Ophthalmol. 2009 Jan. 93(1):83-7. [Medline].

  56. de Carvalho VO, Abagge KT, Giraldi S, Kamoi TO, Assahide MK, Fillus Neto J, et al. Behçet disease in a child--emphasis on cutaneous manifestations. Pediatr Dermatol. 2007 Sep-Oct. 24(5):E57-62. [Medline].

  57. Kesen MR, Goldstein DA, Tessler HH. Uveitis associated with pediatric behçet disease in the american midwest. Am J Ophthalmol. 2008 Dec. 146(6):819-27.e2. [Medline].

  58. Uluduz D, Kürtüncü M, Yapici Z, Seyahi E, Kasapçopur Ö, Özdogan H, et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology. 2011 Nov 22. 77(21):1900-5. [Medline].

  59. Metreau-Vastel J, Mikaeloff Y, Tardieu M, Koné-Paut I, Tran TA. Neurological involvement in paediatric Behçet's disease. Neuropediatrics. 2010 Oct. 41(5):228-34. [Medline].

  60. Seyahi E, Melikoglu M, Akman C, Hamuryudan V, Ozer H, Hatemi G, et al. Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients. Medicine (Baltimore). 2012 Jan. 91(1):35-48. [Medline].

  61. Mizuki N, Inoko H, Ohno S. Molecular genetics (HLA) of Behcet's disease. Yonsei Med J. 1997 Dec. 38(6):333-49. [Medline].

  62. Takeuchi M, Kastner DL, Remmers EF. The immunogenetics of Behçet's disease: A comprehensive review. J Autoimmun. 2015 Nov. 64:137-48. [Medline].

  63. Nishiyama M, Takahashi M, Manaka K, Suzuki S, Saito M, Nakae K. Microsatellite polymorphisms of the MICA gene among Japanese patients with Behcet's disease. Can J Ophthalmol. 2006 Apr. 41(2):210-5. [Medline].

  64. Atagunduz P, Ergun T, Direskeneli H. MEFV mutations are increased in Behcet's disease (BD) and are associated with vascular involvement. Clin Exp Rheumatol. 2003 Jul-Aug. 21(4 Suppl 30):S35-7. [Medline].

  65. Imirzalioglu N, Dursun A, Tastan B, Soysal Y, Yakicier MC. MEFV gene is a probable susceptibility gene for Behcet's disease. Scand J Rheumatol. 2005. 34(1):56-8. [Medline].

  66. Park K, Kim N, Nam J, Bang D, Lee ES. Association of TNFA promoter region haplotype in Behcet's Disease. J Korean Med Sci. 2006 Aug. 21(4):596-601. [Medline].

  67. Baranathan V, Stanford MR, Vaughan RW, Kondeatis E, Graham E, Fortune F. The association of the PTPN22 620W polymorphism with Behcet's disease. Ann Rheum Dis. 2007 Nov. 66(11):1531-3. [Medline].

  68. Serwold T, Gonzalez F, Kim J, Jacob R, Shastri N. ERAAP customizes peptides for MHC class I molecules in the endoplasmic reticulum. Nature. 2002 Oct 3. 419(6906):480-3. [Medline].

  69. Gül A. Genetics of Behçet's disease: lessons learned from genomewide association studies. Curr Opin Rheumatol. 2014 Jan. 26(1):56-63. [Medline].

  70. Park SR, Park KS, Park YJ, Bang D, Lee ES. CD11a, CD11c, and CD18 gene polymorphisms and susceptibility to Behçet's disease in Koreans. Tissue Antigens. 2014 Oct. 84(4):398-404. [Medline].

  71. Hameed AF, Jaradat S, Al-Musawi BM, Sharquie K, Ibrahim MJ, Hayani RK, et al. Association of Higher Defensin β-4 Genomic Copy Numbers with Behçet's Disease in Iraqi Patients. Sultan Qaboos Univ Med J. 2015 Nov. 15 (4):e491-5. [Medline].

  72. Sohn S. Etiopathology of Behcet's disease: herpes simplex virus infection and animal model. Yonsei Med J. 1997 Dec. 38(6):359-64. [Medline].

  73. The Behcet's Disease Research Committee of Japan. Skin hypersensitivity to streptococcal antigens and the induction of systemic symptoms by the antigens in Behçet's disease--a multicenter study. J Rheumatol. 1989 Apr. 16(4):506-11. [Medline].

  74. Mumcu G, Inanc N, Aydin SZ, Ergun T, Direskeneli H. Association of salivary S. mutans colonisation and mannose-binding lectin deficiency with gender in Behçet's disease. Clin Exp Rheumatol. 2009 Mar-Apr. 27(2 Suppl 53):S32-6. [Medline].

  75. Calgüneri M, Ertenli I, Kiraz S, Erman M, Celik I. Effect of prophylactic benzathine penicillin on mucocutaneous symptoms of Behçet's disease. Dermatology. 1996. 192(2):125-8. [Medline].

  76. Apan TZ, Gürsel R, Dolgun A. Increased seropositivity of Helicobacter pylori cytotoxin-associated gene-A in Behçet's disease. Clin Rheumatol. 2007 Jun. 26(6):885-9. [Medline].

  77. Takeno M, Kariyone A, Yamashita N, Takiguchi M, Mizushima Y, Kaneoka H, et al. Excessive function of peripheral blood neutrophils from patients with Behçet's disease and from HLA-B51 transgenic mice. Arthritis Rheum. 1995 Mar. 38(3):426-33. [Medline].

  78. Harrington LE, Mangan PR, Weaver CT. Expanding the effector CD4 T-cell repertoire: the Th17 lineage. Curr Opin Immunol. 2006 Jun. 18(3):349-56. [Medline].

  79. Ozyurt K, Celik A, Sayarlioglu M, Colgecen E, Inci R, Karakas T, et al. Serum Th1, Th2 and Th17 cytokine profiles and alpha-enolase levels in recurrent aphthous stomatitis. J Oral Pathol Med. 2014 Oct. 43(9):691-5. [Medline].

  80. Qi J, Yang Y, Hou S, Qiao Y, Wang Q, Yu H, et al. Increased Notch pathway activation in Behçet's disease. Rheumatology (Oxford). 2014 May. 53(5):810-20. [Medline].

  81. Bouali E, Kaabachi W, Hamzaoui A, Hamzaoui K. Interleukin-37 expression is decreased in Behçet's disease and is associated with inflammation. Immunol Lett. 2015 Oct. 167 (2):87-94. [Medline].

  82. Hamzaoui K, Hamzaoui A, Guemira F, Bessioud M, Hamza M, Ayed K. Cytokine profile in Behçet's disease patients. Relationship with disease activity. Scand J Rheumatol. 2002. 31(4):205-10. [Medline].

  83. Zhou ZY, Chen SL, Shen N, Lu Y. Cytokines and Behcet's disease. Autoimmun Rev. 2012 Aug. 11(10):699-704. [Medline].

  84. Lightman S, Taylor SR, Bunce C, Longhurst H, Lynn W, Moots R, et al. Pegylated interferon-a-2b reduces corticosteroid requirement in patients with Behçet's disease with upregulation of circulating regulatory T cells and reduction of Th17. Ann Rheum Dis. 2014 Sep 30. [Medline].

  85. Fujimori K, Oh-i K, Takeuchi M, Yamakawa N, Hattori T, Kezuka T, et al. Circulating neutrophils in Behçet disease is resistant for apoptotic cell death in the remission phase of uveitis. Graefes Arch Clin Exp Ophthalmol. 2008 Feb. 246(2):285-90. [Medline].

  86. Lew W, Chang JY, Jung JY, Bang D. Increased expression of interleukin-23 p19 mRNA in erythema nodosum-like lesions of Behcet's disease. Br J Dermatol. 2008 Mar. 158(3):505-11. [Medline].

  87. Cekmen M, Evereklioglu C, Er H, et al. Vascular endothelial growth factor levels are increased and associated with disease activity in patients with Behcet's syndrome. Int J Dermatol. 2003 Nov. 42(11):870-5. [Medline].

  88. Kose O, Arca E, Akgul O, Erbil K. The levels of serum neopterin in Behcet's disease--objective marker of disease activity. J Dermatol Sci. 2006 May. 42(2):128-30. [Medline].

  89. Meguro A, Ota M, Katsuyama Y, et al. Association of the toll-like receptor 4 gene polymorphisms with Behcet's disease. Ann Rheum Dis. 2008 May. 67(5):725-7. [Medline].

  90. Oktayoglu P, Mete N, Caglayan M, Bozkurt M, Bozan T, Em S, et al. Elevated serum levels of calprotectin (MRP8/MRP14) in patients with Behçet's disease and its association with disease activity and quality of life. Scand J Clin Lab Invest. 2015 Apr. 75 (2):106-12. [Medline].

  91. Keskin D, Keskin G, Inal A, Ozisik L. Serum angiostatin levels in patients with Behçet's disease: does angiogenesis play a role in the pathogenesis of Behçet's disease?. Acta Clin Belg. 2014 Aug. 69(4):246-50. [Medline].

  92. Kayikçioglu M, Aksu K, Hasdemir C, Keser G, Turgan N, Kültürsay H, et al. Endothelial functions in Behçet's disease. Rheumatol Int. 2006 Feb. 26(4):304-8. [Medline].

  93. Delunardo F, Conti F, Margutti P, et al. Identification and characterization of the carboxy-terminal region of Sip-1, a novel autoantigen in Behcet's disease. Arthritis Res Ther. 2006. 8(3):R71. [Medline].

  94. Leiba M, Seligsohn U, Sidi Y, Harats D, Sela BA, Griffin JH, et al. Thrombophilic factors are not the leading cause of thrombosis in Behçet's disease. Ann Rheum Dis. 2004 Nov. 63(11):1445-9. [Medline]. [Full Text].

  95. Menashi S, Tribout B, Dosquet C, et al. Strong association between plasma thrombomodulin and pathergy test in Behcet disease. Ann Rheum Dis. 2008 Jun. 67(6):892-3. [Medline].

  96. Espinosa G, Font J, Tàssies D, Vidaller A, Deulofeu R, López-Soto A, et al. Vascular involvement in Behçet's disease: relation with thrombophilic factors, coagulation activation, and thrombomodulin. Am J Med. 2002 Jan. 112(1):37-43. [Medline].

  97. Usküdar O, Erdem A, Demiroglu H, Dikmenoglu N. Decreased erythrocyte deformability in Behçet's disease. Clin Hemorheol Microcirc. 2005. 33(2):89-94. [Medline].

  98. Akar S, Ozcan MA, Ates H, Gürler O, Alacacioglu I, Ozsan GH, et al. Circulated activated platelets and increased platelet reactivity in patients with Behçet's disease. Clin Appl Thromb Hemost. 2006 Oct. 12(4):451-7. [Medline].

  99. Alan S, Ulgen MS, Akdeniz S, Alan B, Toprak N. Intima-media thickness and arterial distensibility in Behçet's disease. Angiology. 2004 Jul-Aug. 55(4):413-9. [Medline].

  100. Merashli M, Ster IC, Ames PR. Subclinical atherosclerosis in Behcet's disease: A systematic review and meta-analysis. Semin Arthritis Rheum. 2015 Jul 4. [Medline].

  101. Arimoto J, Endo H, Kato T, Umezawa S, Fuyuki A, Uchiyama S, et al. Clinical value of capsule endoscopy for detecting small bowel lesions in patients with intestinal Behçet's disease. Dig Endosc. 2015 Sep 29. [Medline].

  102. Seo J, Lee M, Choi MJ, Zheng Z, Cho A, Bang D, et al. Predictive value of bone scintigraphy for the detection of joint involvement in Behçet's disease: Dermatologists' perspectives. Eur J Dermatol. 2015 Oct 1. 25 (5):477-82. [Medline].

  103. Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A, et al. Management of Behçet disease: a systematic literature review for the European League Against Rheumatism evidence-based recommendations for the management of Behçet disease. Ann Rheum Dis. 2009 Oct. 68(10):1528-34. [Medline].

  104. Lee JH, Jung JY, Bang D. The efficacy of topical 0.2% hyaluronic acid gel on recurrent oral ulcers: comparison between recurrent aphthous ulcers and the oral ulcers of Behcet's disease. J Eur Acad Dermatol Venereol. 2008 May. 22(5):590-5. [Medline].

  105. Sfikakis PP. Behcet's disease: a new target for anti-tumour necrosis factor treatment. Ann Rheum Dis. 2002 Nov. 61 Suppl 2:ii51-3. [Medline].

  106. Green J, Upjohn E, McCormack C, Zeldis J, Prince HM. Successful treatment of Behcet's disease with lenalidomide. Br J Dermatol. 2008 Jan. 158(1):197-8. [Medline].

  107. Botsios C, Sfriso P, Furlan A, Punzi L, Dinarello CA. Resistant Behcet disease responsive to anakinra. Ann Intern Med. 2008 Aug 19. 149(4):284-6. [Medline].

  108. Yildirim B, Ozturk M, Unal S. The anti-Helicobater pylori antibiotherapy for the treatment of recurrent oral aphthous ulcers in a patient with Behcet's syndrome. Rheumatol Int. 2009. 29:477-478.

  109. Pipitone N, Olivieri I, Cantini F, Triolo G, Salvarani C. New approaches in the treatment of Adamantiades-Behçet's disease. Curr Opin Rheumatol. 2006 Jan. 18(1):3-9. [Medline].

  110. Mochizuki M, Masuda K, Sakane T, Inaba G, Ito K, Kogure M, et al. A multicenter clinical open trial of FK 506 in refractory uveitis, including Behçet's disease. Japanese FK 506 Study Group on Refractory Uveitis. Transplant Proc. 1991 Dec. 23(6):3343-6. [Medline].

  111. Appenzeller S, Hazel E. Pentoxifylline for the treatment of anterior uveitis in Behcet's disease: possible alternative for TNF blockers. Rheumatol Int. 2011 Nov. 31(11):1511-3. [Medline].

  112. Katsiari CG, Theodossiadis PG, Kaklamanis PG, Markomichelakis NN, Sfikakis PP. Successful long-term treatment of refractory Adamantiades-Behcet's disease (ABD) with infliximab: report of two patients. Adv Exp Med Biol. 2003. 528:551-5. [Medline].

  113. Ohno S, Nakamura S, Hori S, et al. Efficacy, safety, and pharmacokinetics of multiple administration of infliximab in Behcet's disease with refractory uveoretinitis. J Rheumatol. 2004 Jul. 31(7):1362-8. [Medline].

  114. Tugal-Tutkun I, Mudun A, Urgancioglu M, et al. Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroids in Behcet's disease: an open-label trial. Arthritis Rheum. 2005 Aug. 52(8):2478-84. [Medline].

  115. Olivieri I, Latanza L, Siringo S, Peruz G, Di Iorio V. Successful treatment of severe Behcet's disease with infliximab in an Italian Olympic athlete. J Rheumatol. 2008 May. 35(5):930-2. [Medline].

  116. Borhani Haghighi A, Safari A, Nazarinia MA, Habibagahi Z, Shenavandeh S. Infliximab for patients with neuro-Behcet's disease: case series and literature review. Clin Rheumatol. 2011 Jul. 30(7):1007-12. [Medline].

  117. Ugras M, Ertem D, Celikel C, Pehlivanoglu E. Infliximab as an alternative treatment for Behcet disease when other therapies fail. J Pediatr Gastroenterol Nutr. 2008 Feb. 46(2):212-5. [Medline].

  118. Estrach C, Mpofu S, Moots RJ. Behcet's syndrome: response to infliximab after failure of etanercept. Rheumatology (Oxford). 2002 Oct. 41(10):1213-4. [Medline].

  119. Capella MJ, Foster CS. Long-term efficacy and safety of infliximab in the treatment of Behçet's disease. Ocul Immunol Inflamm. 2012 Jun. 20(3):198-202. [Medline].

  120. van Laar JA, Missotten T, van Daele PL, Jamnitski A, Baarsma GS, van Hagen PM. Adalimumab: a new modality for Behcet's disease?. Ann Rheum Dis. 2007 Apr. 66(4):565-6. [Medline].

  121. Godeau P, Wechsler B, Maaouni A, Fagard M, Herreman G. [Cardiovascular involvement in Behçet's disease. (author's transl)]. Ann Dermatol Venereol. 1980 Aug-Sep. 107 (8-9):741-7. [Medline].

  122. Geri G, Wechsler B, Thi Huong du L, Isnard R, Piette JC, Amoura Z, et al. Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature. Medicine (Baltimore). 2012 Jan. 91 (1):25-34. [Medline].

  123. Fekih M, Fennira S, Ghodbane L, Zaouali RM. [Intracardiac thrombosis: unusual complication of Behcet's disease]. Tunis Med. 2004 Aug. 82 (8):785-90. [Medline].

  124. Vallet H, Riviere S, Sanna A, et al. Efficacy of anti-TNF alpha in severe and/or refractory Behçet's disease: Multicenter study of 124 patients. J Autoimmun. 2015 Aug. 62:67-74. [Medline].

Minor aphthous ulcer.
Major aphthous ulcer.
Herpetiform oral ulcer.
A characteristic genital ulcer on scrotum.
A single ulcer on vulva.
Erythema nodosum–like lesions on skin.
Papulopustular eruptions.
Sweet syndrome–like lesion.
Typical positive pathergy reaction at injection site.
Ocular involvement showing posterior uveitis.
CT angiogram showing pulmonary artery aneurysm in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Acneiform lesions in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Superficial thrombophlebitis in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Postphlebitic limb in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Acneiform lesions in a patient with Behçet disease (an inflamed nodule and noninflammatory comedones). Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Radial artery aneurysm with its effect distally on index finger in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.