Dermatologic Aspects of Behcet Disease Differential Diagnoses

  • Author: Marjan Yousefi, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Dec 23, 2010
 
 
 
Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

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.

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Medical Dermatology Society, Michigan Dermatological Society, Michigan State Medical Society, Photomedicine Society, Society for Investigative Dermatology, and Wound Healing Society

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

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

Disclosure: Nothing to disclose.

Warren R Heymann, MD  Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey

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

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

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

Disclosure: Nothing to disclose.

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

  2. Boe J, Dalgaard JB, Scott D. Mucocutaneous-ocular syndrome with intestinal involvement; a clinical and pathological study of four fatal cases. Am J Med. Dec 1958;25(6):857-67. [Medline].

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

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

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

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

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

  8. Sechas MN, Liapis CD, Gougoulakis AG, Mandrekas DP, Fotiadis CI, Vaiopoulos GA. Vascular manifestations of Behcet's disease. Int Angiol. Jul-Sep 1989;8(3):145-50. [Medline].

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

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

  11. Uzun S, Alpsoy E, Durdu M, Akman A. The clinical course of Behcet's disease in pregnancy: a retrospective analysis and review of the literature. J Dermatol. Jul 2003;30(7):499-502. [Medline].

  12. Kansu E, Deglin S, Cantor RI, Burke JF Jr, Cho SY, Cathart RT. The expanding spectrum of Behcet syndrome: a case with renal involvement. JAMA. Apr 25 1977;237(17):1855-6. [Medline].

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

  14. 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. Apr 2006;41(2):210-5. [Medline].

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

  16. 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].

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

  18. 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. Nov 2007;66(11):1531-3. [Medline].

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

  20. Duzgun N, Ayaslioglu E, Tutkak H, Aydintug OT. Cytokine inhibitors: soluble tumor necrosis factor receptor 1 and interleukin-1 receptor antagonist in Behcet's disease. Rheumatol Int. Jan 2005;25(1):1-5. [Medline].

  21. 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. Aug 2003;30(8):602-7. [Medline].

  22. 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. Mar 2008;158(3):505-11. [Medline].

  23. 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. Nov 2003;42(11):870-5. [Medline].

  24. 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. May 2006;42(2):128-30. [Medline].

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

  26. 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].

  27. Esmat S, El Sherif H, Anwar S, Fahmy I, Elmenyawi M, Shaker O. Lipoprotein (a) and nitrites in Behcet's disease: relationship with disease activity and vascular complications. Eur J Dermatol. Jan-Feb 2006;16(1):67-71. [Medline].

  28. Ricart JM, Vaya A, Todoli J, et al. Thrombophilic risk factors and homocysteine levels in Behcet's disease in eastern Spain and their association with thrombotic events. Thromb Haemost. Apr 2006;95(4):618-24. [Medline].

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

  30. Ricart JM, Ramon LA, Vaya A, et al. Fibrinolytic inhibitor levels and polymorphisms in Behcet disease and their association with thrombosis. Br J Haematol. May 2008;141(5):716-9. [Medline].

  31. 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. May 2008;22(5):590-5. [Medline].

  32. Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A. 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. Oct 2009;68(10):1528-34. [Medline].

  33. Kotake S, Higashi K, Yoshikawa K, Sasamoto Y, Okamoto T, Matsuda H. Central nervous system symptoms in patients with Behçet disease receiving cyclosporine therapy. Ophthalmology. Mar 1999;106(3):586-9. [Medline].

  34. Kötter I, Günaydin I, Batra M, Vonthein R, Stübiger N, Fierlbeck G. CNS involvement occurs more frequently in patients with Behçet's disease under cyclosporin A (CSA) than under other medications--results of a retrospective analysis of 117 cases. Clin Rheumatol. Jul 2006;25(4):482-6. [Medline].

  35. Kato Y, Numaga J, Kato S, Kaburaki T, Kawashima H, Fujino Y. Central nervous system symptoms in a population of Behçet's disease patients with refractory uveitis treated with cyclosporine A. Clin Experiment Ophthalmol. Oct 2001;29(5):335-6. [Medline].

  36. Alpsoy E, Durusoy C, Yilmaz E, et al. Interferon alfa-2a in the treatment of Behcet disease: a randomized placebo-controlled and double-blind study. Arch Dermatol. Apr 2002;138(4):467-71. [Medline].

  37. Kotter I, Zierhut M, Eckstein AK, et al. Human recombinant interferon alfa-2a for the treatment of Behcet's disease with sight threatening posterior or panuveitis. Br J Ophthalmol. Apr 2003;87(4):423-31. [Medline].

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

  39. 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.

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

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

  42. 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].

  43. 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. Jul 2004;31(7):1362-8. [Medline].

  44. 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. Aug 2005;52(8):2478-84. [Medline].

  45. 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. May 2008;35(5):930-2. [Medline].

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

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

  48. 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. Apr 2007;66(4):565-6. [Medline].

  49. [Best Evidence] Melikoglu M, Fresko I, Mat C, et al. Short-term trial of etanercept in Behcet's disease: a double blind, placebo controlled study. J Rheumatol. Jan 2005;32(1):98-105. [Medline].

  50. Cho MY, Lee SH, Bang D, Lee S. Epidemiologic findings of Behcet's syndrome. Korean J Dermatol. 1988;26:320-9.

  51. Gharibdoost F, Davatchi F, Shahram F, et al. Clinical manifestations of Behcet's disease in Iran. Analysis of 2176 cases. In: Godeau P, Wechsler B, eds. Proceedings of the 6th International Conference on Behcet's Disease. Amsterdam, The Netherlands: Elsevier Science; 1993:153-8.

  52. Ghate JV, Jorizzo JL. Behcet's disease and complex aphthosis. J Am Acad Dermatol. Jan 1999;40(1):1-18; quiz 19-20. [Medline].

  53. Kaklamani VG, Vaiopoulos G, Kaklamanis PG. Behcet's Disease. Semin Arthritis Rheum. Feb 1998;27(4):197-217. [Medline].

  54. Sakane T, Suzuki N, Nagafuchi H. Etiopathology of Behcet's disease: immunological aspects. Yonsei Med J. Dec 1997;38(6):350-8. [Medline].

  55. Saylan T, Mat C, Fresko I, Melikoglu M. Behcet's disease in the Middle East. Clin Dermatol. Mar-Apr 1999;17(2):209-23; discussion 105-6. [Medline].

  56. Saylan T, Urgancioglu M, Akarcay K, Bozan G. General data of the patients with Behcet's disease. In: Dilsen N, Konice M, Ovul C, eds. Proceedings of the 2nd International Symposium on Behcet's Disease. Amsterdam, The Netherlands: Elsevier Science; 1977:130-2.

  57. Wong RC, Ellis CN, Diaz LA. Behcet's disease. Int J Dermatol. Jan-Feb 1984;23(1):25-32. [Medline].

  58. Yazici H, Yurdakul S, Hamuryudan V. Behcet's syndrome. Curr Opin Rheumatol. Jan 1999;11(1):53-7. [Medline].

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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.
 
 
 
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