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The Mucocutaneous Manifestations and Pathergy Reaction in Behçet Syndrome

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Behçet Syndrome

Abstract

The diagnosis of Behçet syndrome (BS) is based on clinical features. There is no specific diagnostic test. Mucocutaneous lesions like recurrent aphthous stomatitis, genital ulcers, and various cutaneous lesions are its foremost manifestations. The presence of erythema nodosum-like lesions, pseudofolliculitis, papulopustular lesions, acneiform nodules, superficial thrombophlebitis, and cutaneous hyperreactivity (pathergy) are included in the criteria of the International Study Group for Behçet’s disease even though there is debate that the presence of some of these lesions in any one patient is coincidental. Mucocutaneous lesions in BS may differ in both clinical and histopathologic appearance during the disease course, and the common underlying pathology is a vasculitis affecting blood vessels of all sizes. Different therapeutic approaches including immunomodulatory or immunosuppressive drugs have been introduced with increasing trend of the newer biological agents.

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References

  1. Behçet H. Ueber rezidivierende aphtose durch ein virus verursachte geschwuere am mund, am auge und an den genitalien. Dermatol Wochenschr. 1937;105:1152.

    Google Scholar 

  2. Yazici H, Yurdakul S, Hamuryudan V. Behçet’s syndrome. In: Maddison PJ, Isenberg DA, Woo P, Glass DN, editors. Oxford textbook of rheumatology. 2nd ed. Oxford: Oxford University Press; 1998. p. 1394–402.

    Google Scholar 

  3. Zunt SL. Recurrent aphthous stomatitis. Dermatol Clin. 2003;21:33–9.

    Article  PubMed  Google Scholar 

  4. Gürler A, Boyvat A, Tursen U. Clinical manifestations of Behçet’s disease. An analysis of 2147 patients. Yonsei Med J. 1997;38:423–7.

    Article  PubMed  Google Scholar 

  5. Alpsoy E, Dönmez L, Önder M, et al. Clinical features and natural course of Behçet’s disease in 661 cases. A multicenter study. Br J Dermatol. 2007;157:901–6.

    Article  CAS  PubMed  Google Scholar 

  6. Saylan T, Mat C, Fresko I, Melikoğlu M. Behçet’s disease in the middle-east. Clin Dermatol. 1999;17:209–23.

    Article  CAS  PubMed  Google Scholar 

  7. Bang D, Hur W, Lee ES, Lee S. Prognosis and clinical relevance of recurrent oral ulceration in Behçet’s disease. J Dermatol. 1995;22:926–9.

    Article  CAS  PubMed  Google Scholar 

  8. McCarthy MA, Garton RA, Jorizzo JL. Complex aphthosis and Behçet’s disease. Dermatol Clin. 2003;21:41–8.

    Article  Google Scholar 

  9. Rogers RS III. Recurrent aphthous stomatitis in diagnosis of Behçet’s disease. Yonsei Med J. 1997;38:370–9.

    Article  PubMed  Google Scholar 

  10. Letsinger JA, MacCarty MA, Jorizzo JL. Complex aphthosis: a large case series with evaluation algorithm and therapeutic ladder from topicals to thalidomide. J Am Acad Dermatol. 2005;52:500–8.

    Article  PubMed  Google Scholar 

  11. Ifeacho SN, Malhi G, Hamburger M. Recurrent aphtous stomatitis and Behçet’s disease: is there a link? XI International Congress on BD. Clin Exp Rheumatol. 2004;22(4):101.

    Google Scholar 

  12. Main DM, Chamberlain MA. Clinical differentiation of oral ulceration in Behçet’s disease. Br J Rheumatol. 1992;31:767–70.

    Article  CAS  PubMed  Google Scholar 

  13. International Study Group for Behçet’s Disease. Criteria for diagnosis of Behçet’s disease. Lancet. 1990;335:1078–80.

    Google Scholar 

  14. Hamuryudan V, Mat C, Saip S, Ozyazgan Y, Siva A, Yurdakul S, et al. Thalidomide in the treatment of the mucocutaneous lesions of the Behçet’s syndrome. A randomized, double-blind, placebo-controlled trial. Ann Intern Med. 1998;128(6):443–50.

    Article  CAS  PubMed  Google Scholar 

  15. Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, et al. Short-term trial of etanercept in Behçet’s disease: a double blind, placebo controlled study. J Rheumatol. 2005;32:98–105.

    CAS  PubMed  Google Scholar 

  16. Yurdakul S, Mat C, Tüzün Y, Ozyazgan Y, Hamuryudan V, Uysal O, et al. A double-blind trial of colchicine in Behçet’s syndrome. Arthritis Rheum. 2001;44(11):2686–92.

    Article  CAS  PubMed  Google Scholar 

  17. Cosgun S, Seyahi E, Mat C, Yazici H. Female Behçet’s syndrome patients have more severe oral ulceration. XI International congress on Behçet’s disease. Clin Exp Rheumatol. 2004;22:86.

    Google Scholar 

  18. Krause I, Rosen Y, Kaplan I, Milo G, Guedj D, Molad Y, Weinberger A. Recurrent aphthous stomatitis in Behçet’s disease: clinical features and correlation with systemic disease expression and severity. J Oral Pathol Med. 1999;28(5):193–6.

    Article  CAS  PubMed  Google Scholar 

  19. Mumcu G, Sur H, Inanc N, Karacayli U, Cimilli H, Sisman N, et al. A composite index for determining the impact of oral ulcer activity in Behçet’s disease and recurrent aphthous stomatitis. J Oral Pathol Med. 2009;38(10):785–91.

    Article  CAS  PubMed  Google Scholar 

  20. Sharquie KE, Al-Araji A, Hatem A. Oral pathergy test in Behçet’s disease. Br J Dermatol. 2002;146(1):168–9.

    Article  CAS  PubMed  Google Scholar 

  21. Soy M, Erken E, Konca K, Ozbek S. Smoking and Behçet’s disease. Clin Rheumatol. 2000;19(6):508–9.

    Article  CAS  PubMed  Google Scholar 

  22. Kaklamani VG, Tzonou A, Markomichelakis N, Papazoglou S, Kaklamanis PG. The effect of smoking on the clinical features of Adamantiades-Behçet’s disease. Adv Exp Med Biol. 2003;528:323–7.

    Article  PubMed  Google Scholar 

  23. Mat C, Goksugur N, Ergin B, Yurdakul S, Yazici H. The frequency of scarring after genital ulcers in Behçet’s syndrome: a prospective study. Int J Dermatol. 2006;45:554–6.

    Article  PubMed  Google Scholar 

  24. Bang D, Lee ES, Sohn S. Behçet’s disease: a guide to its clinical understanding. Heidelberg: Springer; 2001.

    Book  Google Scholar 

  25. Alpsoy E, Zouboulis CC, Ehrlich GE. Mucocutaneous lesions of Behçet’s disease. Yonsei Med J. 2007;48:573–85.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Lee ES, Bang D, Lee S. Dermatologic manifestation of Behçet’s disease. Yonsei Med J. 1997;38:380–9.

    Article  CAS  PubMed  Google Scholar 

  27. Lin P, Liang G. Behçet’s disease: recommendation for clinical management of mucocutaneous lesions. J Clin Rheumatol. 2006;12:282–6.

    Article  PubMed  Google Scholar 

  28. Kim B, LeBoit PE. Histopathologic features of erythema nodosum-like lesion in Behçet’s disease: a comparison with erythema nodosum focusing on the role of vasculitis. Am J Dermatopathol. 2000;22:379–90.

    Article  CAS  PubMed  Google Scholar 

  29. Demirkesen C, Tuzuner N, Mat C, Senocak M, Buyukbabani N, Tuzun Y, et al. Clinicopathologic evaluation of nodular cutaneous lesions of Behçet’s syndrome. Am J Clin Pathol. 2001;116:341–6.

    Article  CAS  PubMed  Google Scholar 

  30. Yazici H, Tüzün Y, Pazarli H, Yurdakul S, Ozyazgan Y, Ozdoğan H, et al. Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis. 1984;43(6):783–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Mat CM, Demirkesen C, Melikoglu M, Yazici H. Behçet’s syndrome. In: Sarzi-Puttuni P, Doria A, Girolomoni G, Kuhn A, editors. The skin and autoimmune disease. Amsterdam: Elsevier; 2006. p. 185–206.

    Chapter  Google Scholar 

  32. Lee H, Kim SH, Lee SW, Zheng Z, Bang D, Kim DY. A case of extranodal natural killer/T-cell lymphoma mimicking refractory Behçet’s disease. Acta Derm Venereol. 2015;95(4):491–2.

    Article  PubMed  Google Scholar 

  33. Kuzu MA, Ozaslan C, Köksoy C, Gürler A, Tüzüner A. Vascular involvement in Behçet’s disease: 8-year audit. World J Surg. 1994;18(6):948–53.

    Article  CAS  PubMed  Google Scholar 

  34. 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(12):1693–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Kucukoglu S, Tunc R, Cetinkaya F, Demirkesen C, Mat C, Yazici H. The importance of cutaneous ultrasonography on the differentiation of nodular skin lesions seen in patients with Behçet’s disease. Yonsei Med J. 2000;41(3):S40.

    Google Scholar 

  36. Seyahi E, Yurdakul S. Behçet’s syndrome and thrombosis. Mediterr J Hematol Infect Dis. 2011;3(1):e2011026.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Jorizzo JL, Abernethy JL, White WL, Mangelsdorf HC, Zouboulis CC, Sarica R, et al. Mucocutaneous criteria for the diagnosis of Behçet’s disease: an analysis of clinicopathologic data from multiple international centers. J Am Acad Dermatol. 1995;32(6):968–76.

    Article  CAS  PubMed  Google Scholar 

  38. Luelmo-Aguilar J, Santandreu MS. Folliculitis: recognition and management. Am J Clin Dermatol. 2004;5(5):301–10.

    Article  PubMed  Google Scholar 

  39. Yazici H, Hekim N, Tüzün Y, Serdaroglu S, Kotogyan A, Öz F, et al. Sex factor and Behçet’s syndrome. In: Lehner T, Barnes CG, editors. Recent advances in Behçet’s disease. London: Royal Society of Medicine Services; 1986. p. 205–6.

    Google Scholar 

  40. Ergun T, Gurbuz O, Dogusoy G, Mat C, Yazici H. Histopathologic features of the spontaneous pustular lesions of Behçet’s syndrome. Int J Dermatol. 1998;37:194–6.

    Article  CAS  PubMed  Google Scholar 

  41. Alpsoy E, Aktekin M, Er H, Durusoy C, Yilmaz E. A randomized, controlled and blinded study of papulopustular lesions in Turkish Behçet’s patients. Int J Dermatol. 1998;37(11):839–42.

    Article  CAS  PubMed  Google Scholar 

  42. Kutlubay Z, Ozguler Y, Hatemi G, Tascilar K, Mat C, Yazici H. Papulopustular lesions according to age, sex and body parts in Behçet’s syndrome patients compared health populations and diseased control. Ann Rheum Dis. 2017;76:619.

    Google Scholar 

  43. Kutlubay Z, Mat CM, Aydin Ö, Demirkesen C, Calay Ö, Engın B, et al. Histopathological and clinical evaluation of papulopustular lesions in Behçet’s disease. Clin Exp Rheumatol. 2015;33(6. Suppl 94):101–6.

    Google Scholar 

  44. Kligman AM. An overview of acne. J Invest Dermatol. 1974;62(3):268–87.

    Article  CAS  PubMed  Google Scholar 

  45. Kligman AM. Postadolescent acne in women. Cutis. 1991;48(1):75–7.

    CAS  PubMed  Google Scholar 

  46. Goulden V, McGeown CH, Cunliffe WJ. The familial risk of adult acne: a comparison between first-degree relatives of affected and unaffected individuals. Br J Dermatol. 1999;141(2):297–300.

    Article  CAS  PubMed  Google Scholar 

  47. Thiboutot D, Gilliland K, Light J, Lookingbill D. Androgen metabolism in sebaceous glands from subjects with and without acne. Arch Dermatol. 1999;135(9):1041–5.

    Article  CAS  PubMed  Google Scholar 

  48. Yazici H, Mat C, Deniz S, Iscimen A, Yurdakul S, Tuzun Y, et al. Sebum production is increased in Behçet’s syndrome and even more so in rheumatoid arthritis. Clin Exp Rheumatol. 1987;5(4):371–4.

    CAS  PubMed  Google Scholar 

  49. Alpsoy E, Elpek GO, Yilmaz F, Ciftcioglu MA, Akman A, Uzun S, et al. Androgen receptor levels of oral and genital ulcers and skin pathergy test in patients with Behçet’s disease. Dermatology. 2005;210(1):31–5.

    Article  CAS  PubMed  Google Scholar 

  50. Hatemi G, Bahar H, Uysal S, Mat C, Gogus F, Masatlioglu S, et al. The pustular skin lesions in Behçet’s syndrome are not sterile. Ann Rheum Dis. 2004;63(11):1450–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Inanc N, Mumcu G, Birtas E, Elbir Y, Yavuz S, Ergun T, et al. Serum mannose-binding lectin levels are decreased in Behçet’s disease and associated with disease severity. J Rheumatol. 2005;32(2):287–91.

    CAS  PubMed  Google Scholar 

  52. Alpsoy E, Uzun S, Akman A, Acar MA, Memişoglu HR, Başaran E. Histological and immunofluorescence findings of non-follicular papulopustular lesions in patients with Behçet’s disease. J Eur Acad Dermatol Venereol. 2003;17(5):521–4.

    Article  CAS  PubMed  Google Scholar 

  53. Ilknur T, Pabuççuoglu U, Akin C, Lebe B, Gunes AT. Histopathologic and direct immunofluorescence findings of the papulopustular lesions in Behçet’s disease. Eur J Dermatol. 2006;16(2):146–50.

    PubMed  Google Scholar 

  54. Kalkan G, Karadag AS, Astarci HM, Akbay G, Ustun H, Eksioglu M. A histopathological approach: when papulopustular lesions should be in the diagnostic criteria of Behçet’s disease? J Eur Acad Dermatol Venereol. 2009;23(9):1056–60.

    Article  CAS  PubMed  Google Scholar 

  55. Boyvat A, Heper AO, Koçyiğit P, Erekul S, Gürgey E. Can specific vessel-based papulopustular lesions of Behçet’s disease be differentiated from nonspecific follicular-based lesions clinically? Int J Dermatol. 2006;45(7):814–8.

    Article  PubMed  Google Scholar 

  56. Ergun T, Gürbüz O, Dogusoy G, Mat C, Yazici H. Histopathologic features of the spontaneous pustular lesions of Behçet’s syndrome. Int J Dermatol. 1998;37(3):194–6.

    Article  CAS  PubMed  Google Scholar 

  57. 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;60(11):1074–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  58. Jung JY, Kim DY, Bang D. Leg ulcers in Behçet’s disease. Br J Dermatol. 2008;158:172–203.

    Google Scholar 

  59. Oh SH, Lee JH, Shin JU, Bang D. Dermatological features in Behçet’s disease-associated vena cava obstruction. Br J Dermatol. 2008;159:555–60.

    Article  PubMed  Google Scholar 

  60. Golan G, Beeri R, Mevorach D. Henoch–Schonlein purpura-like disease representing a flare of Behçet’s disease. Br J Rheumatol. 1994;33:1198–9.

    Article  CAS  PubMed  Google Scholar 

  61. Park YW, Park JJ, Lee JB, et al. Development of Henoch–Schonlein purpura in a patient with Behçet’s disease presenting with recurrent deep vein thrombosis. Clin Exp Rheumatol. 2007;25:S96–8.

    PubMed  Google Scholar 

  62. Chen KR, Kawahara Y, Miyakawa S, et al. Cutaneous vasculitis in Behçet’s disease: a clinical and histopathologic study of 20 patients. J Am Acad Dermatol. 1997;36:689–96.

    Article  CAS  PubMed  Google Scholar 

  63. Bilic M, Mutasim DF. Neutrophilic eccrine hidradenitis in a patient with Behçet’s disease. Cutis. 2001;68:107–11.

    CAS  PubMed  Google Scholar 

  64. Nijsten TE, Meuleman L, Lambert J. Chronic pruritic neutrophilic eccrine hidradenitis in a patient with Behçet’s disease. Br J Dermatol. 2002;147:797–800.

    Article  CAS  PubMed  Google Scholar 

  65. Mercader-Garcia P, Vilata-Corell JJ, Pardo-Sanchez J, et al. Neutrophilic eccrine hidradenitis in a patient with Behçet’s disease. Acta Derm Venereol. 2003;83:395–6.

    Article  PubMed  Google Scholar 

  66. Cantini F, Salvarani C, Niccoli L, et al. Behçet’s disease with unusual cutaneous lesions. J Rheumatol. 1998;25:2469–72.

    CAS  PubMed  Google Scholar 

  67. Korkmaz C, Aydinli A, Erol N, et al. Widespread nocardiosis in two patients with Behçet’s disease. Clin Exp Rheumatol. 2001;19:459–62.

    CAS  PubMed  Google Scholar 

  68. King R, Crowson AN, Murray E, et al. Acral purpuric papulonodular lesions as a manifestation of Behçet’s disease. Int J Dermatol. 1995;34:190–2.

    Article  CAS  PubMed  Google Scholar 

  69. Aydin F, Senturk N, Yildiz L, et al. Behçet’s disease with unusual cutaneous lesions. J Eur Acad Dermatol Venereol. 2006;20:106–7.

    Article  CAS  PubMed  Google Scholar 

  70. Callen PR. Sweet’s syndrome-a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34.

    Article  Google Scholar 

  71. Magro CM, Crowson AN. Cutaneous manifestations of Behçet’s disease. Int J Dermatol. 1995;34:159–65.

    Article  CAS  PubMed  Google Scholar 

  72. Hui-li S, Zheng-ji H. Study on cutaneous lesions in Behçet’s disease and meanings of relative laboratory parameters. In: Godeau F, Weschler B, editors. Behçet’s disease, proceedings of the sixth international conference on Behçet’s disease. Amsterdam: Excerpta Medica; 1993. p. 325.

    Google Scholar 

  73. Oguz O, Serdaroglu S, Tuzun Y, Erdogan N, Yazici H, Savaskan H. Acute febrile neutrophilic dermatosis associated with Behçet’s syndrome. Int J Dermatol. 1992;31:645–6.

    Google Scholar 

  74. Nelson CA, Stephen S, Ashchyan HJ, James WD, Micheletti RG, Rosenbach M. Neutrophilic dermatoses. Part I. Pathogenesis, sweet syndrome, neutrophilic eccrine hidradenitis. J Am Acad Dermatol. 2018;79(6):987–1006.

    Article  PubMed  Google Scholar 

  75. Azizlerli G, Ozarmagan G, Ovul C, Sarica R, Mustafa SO. A new kind of skin lesion in Behçet’s disease: extragenital ulcers. Acta Derm Venereol. 1992;72:286.

    CAS  PubMed  Google Scholar 

  76. Kutlubay Z, Tüzün Y, Wolf R. Pathergy test as a diagnostic tool. Skinmed. 2017;15(2):97–104.

    PubMed  Google Scholar 

  77. Ozarmagan G, Saylan T, Azizlerli G, Ovul C, Aksungur VL. Re-evaluation of the pathergy test in Behçet’s disease. Acta Derm Venereol. 1991;71:75–6.

    CAS  PubMed  Google Scholar 

  78. Krause I, Molad Y, Mitrani M, Weinberger A. Pathergy reaction in Behçet’s disease: lack of correlation with mucocutaneous manifestations and systemic disease expression. Clin Exp Rheumatol. 2000;18:71–4.

    CAS  PubMed  Google Scholar 

  79. Cakir N, Yazici H, Chamberlain MA, Barnes CG, Yurdakul S, Atasoy S, et al. Response to intradermal injection of monosodium urate crystals in Behçet’s syndrome. Ann Rheum Dis. 1991;50:634–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  80. Yazici H, Tüzün Y, Tanman AB, Yurdakul S, Serdaroglu S, Pazarli H, et al. Male patients with Behçet’s syndrome have stronger pathergy reactions. Clin Exp Rheumatol. 1985;3(2):137–41.

    CAS  PubMed  Google Scholar 

  81. Faezi ST. Pathergy test in Behçet’s disease: diagnostic or prognostic? Clin Exp Rheumatol. 2016;34.(Suppl. 102:S161.

    Google Scholar 

  82. Alpagut U, Ugurlucan M, Dayioglu E. Major arterial involvement and review of Behçet’s disease. Ann Vasc Surg. 2007;21(2):232–9.

    Article  PubMed  Google Scholar 

  83. Tüzün H, Sayin A, Karaözbek Y, Erdağ A, Coskun H, Vural FS. Peripheral aneurysms in Behçet’s disease. Cardiovasc Surg. 1993;1(3):220–4.

    PubMed  Google Scholar 

  84. Tüzün H, Besirli K, Sayin A, Vural FS, Hamuryudan V, Hizli N, et al. Management of aneurysms in Behçet’s syndrome: an analysis of 24 patients. Surgery. 1997;121(2):150–6.

    Article  PubMed  Google Scholar 

  85. Yalcindag FN, Batioglu F. Pathergy-like reaction following intravitreal triamcinolone acetonide injection in a patient with Behçet’s disease. Ocul Immunol Inflamm. 2008;16(4):181–3.

    Article  PubMed  Google Scholar 

  86. 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;43(5):692–700.

    Article  CAS  PubMed  Google Scholar 

  87. Mat MC, Nazarbaghi M, Tüzün Y, Hamuryudan V, Hızlı N, Yurdakul S, et al. Wound healing in Behçet’s syndrome. Int J Dermatol. 1998;37:120–3.

    Article  CAS  PubMed  Google Scholar 

  88. 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.

    CAS  PubMed  Google Scholar 

  89. Dilsen N, Konice M, Aral O, Ocal L, Inanc M, Gül A. Comparative study of the skin pathergy test with blunt and sharp needles in Behçet’s disease: confirmed specificity but decreased sensitivity with sharp needles. Ann Rheum Dis. 1993;52:823–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  90. Fresko I, Yazici H, Bayramicli M, Yurdakul S, Mat C. Effect of surgical cleaning of the skin on the pathergy phenomenon in Behçet’s syndrome. Ann Rheum Dis. 1993;52:619–20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  91. Fresko I, Ozsoy Y, Mat C, Melikoglu M, Tunc R, Yazici H. The response to the intradermal injection to monosodium urate in Behçet’s syndrome and its comparison to the pathergy test. Yonsei Med J. 2000;41(3):S25.

    Google Scholar 

  92. Togashi A, Sanae S, Kaneko F, Nakamura K, Oyama N. Skin prick test with self-saliva in patients with oral aphthoses: a new diagnostic pathergy for Behçet’s disease and recurrent aphthosis. Inflamm Allergy Drug Tagets. 2011;10(3):164–70.

    Article  CAS  Google Scholar 

  93. Scherrer MAR, Rocha VB, Garcia LC. Behçet’s disease: review with emphasis on dermatological aspects. An Bras Dermatol. 2017;92(4):452–64.

    Article  PubMed  PubMed Central  Google Scholar 

  94. Sevim A, Kutlubay Z, Engin B, Serdaroğlu S, Tüzün Y. Pathergy testing: prospective comparison of dermatoscopic evaluation and naked eye examination. In: 2nd international dermatology and cosmetology congress. 15–18 Mar 2017. p. 29–32.

    Google Scholar 

  95. Gul A, Esin S, Dilsen N, Konice M, Wigzell H, Biberfeld P. Immunohistology of skin pathergy reaction in Behçet’s disease. Br J Dermatol. 1995;132:901–7.

    Article  CAS  PubMed  Google Scholar 

  96. Melikoglu M, Uysal S, Krueger JG, Kaplan G, Gogus F, Yazici H, et al. Characterization of the divergent wound-healing responses occurring in the pathergy reaction and normal healthy volunteers. J Immunol. 2006;177:6415–21.

    Article  CAS  PubMed  Google Scholar 

  97. Ben Ahmed M, Houman H, Miled M, Dellagi K, Louzir H. Involvement of chemokines and Th1 cytokines in the pathogenesis of mucocutaneous lesions of Behçet’s disease. Arthritis Rheum. 2004;50:2291–5.

    Article  CAS  PubMed  Google Scholar 

  98. Jorizzo JL, Solomon AR, Cavallo T. Behçet’s syndrome. Immunopathologic and histopathologic assessment of pathergy lesions is useful in diagnosis and follow-up. Arch Pathol Lab Med. 1985;109:747–51.

    CAS  PubMed  Google Scholar 

  99. Gilhar A, Winterstein G, Turani H, Landau J, Etzioni A. Skin hyperreactivity response (pathergy) in Behçet’s disease. J Am Acad Dermatol. 1989;21:547–52.

    Article  CAS  PubMed  Google Scholar 

  100. Haim S, Sobel JD, Friedman-Birnbaum R, Lichtig C. Histological and direct immunofluorescence study of cutaneous hyperreactivity in Behçet’s disease. Br J Dermatol. 1976;95:631–6.

    Article  CAS  PubMed  Google Scholar 

  101. Gilhar A, Haim S, Wolf V, Golan D. Mast cells in Behçet’s disease: ultrastructural and histamine content studies. J Dermatol. 1983;10:185–6.

    Article  CAS  PubMed  Google Scholar 

  102. Budak-Alpdogan T, Demircay Z, Alpdogan O, Direskeneli H, Ergun T, Bayik M, et al. Skin hyperreactivity of Behçet’s patients (pathergy reaction) is also positive in interferon alpha-treated chronic myeloid leukaemia patients, indicating similarly altered neutrophil functions in both disorders. Ann Haematol. 1997;74:45–8.

    Article  CAS  Google Scholar 

  103. Tascilar K, Baran A, Melikoglu M, Gogus F, Hatemi G, Yazici H. Effect of immunosuppressive treatment on skin pathergy reaction in Behçet’s syndrome. Clin Exp Rheumatol. 2008;26(4):30.

    Google Scholar 

  104. Jorizzo JL, Taylor RS, Schmalstieg FC, Solomon AR Jr, Daniels JC, Rudloff HE, et al. Complex aphthosis: a forme fruste of Behçet’s syndrome? J Am Acad Dermatol. 1985;13(1):80–4.

    Article  CAS  PubMed  Google Scholar 

  105. Keogan MT. Clinical ımmunology review series: an approach to the patient with recurrent orogenital ulceration, including Behçet’s syndrome. Clin Exp Immunol. 2009;156(1):1–11.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  106. Rogers RS 3rd. Pseudo-Behçet’s disease. Dermatol Clin. 2003;156(1):49–61.

    Article  Google Scholar 

  107. Hatemi I, Hatemi G, Celik AF, Melikoglu M, Arzuhal N, Mat C, et al. Frequency of pathergy phenomenon and other features of Behçet’s syndrome among patients with inflammatory bowel disease. Clin Exp Rheumatol. 2008;26:S91–5.

    CAS  PubMed  Google Scholar 

  108. Tunc R, Uluhan A, Melikoglu M, Ozyazgan Y, Ozdogan H, Yazici H. A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet’s syndrome. Clin Exp Rheumatol. 2001;19(5 Suppl 24):S45–7.

    CAS  PubMed  Google Scholar 

  109. Jonsson R, Heyden G, Westberg NG, Nyberg G. Oral mucosal lesions in systemic lupus erythematosus – a clinical, histopathological and immunopathological study. J Rheumatol. 1984;11(1):38–42.

    CAS  PubMed  Google Scholar 

  110. Fresko I, Yazici H, Isci H, Yurdakul S. Genital ulceration in patients with systemic lupus erythematosus. Lupus. 1993;2(2):135.

    Article  CAS  PubMed  Google Scholar 

  111. Gül A. Behçet’s disease as an autoinflammatory disorder. Curr Drug Targets Inflamm Allergy. 2005;4(1):81–3.

    Article  PubMed  Google Scholar 

  112. Taylor J, Glenny AM, Walsh T, Brocklehurst P, Riley P, Gorodkin R, et al. Interventions for the management of oral ulcers in Behçet’s disease. Cochrane Database Syst Rev. 2014;25(9):CD011018.

    Google Scholar 

  113. Alpsoy E, Er H, Durusoy C, Yilmaz E. The use of sucralfate suspension in the treatment of oral and genital ulceration of Behçet’s disease: a randomized, placebo-controlled, double-blind study. Arch Dermatol. 1999;135:529–32.

    Article  CAS  PubMed  Google Scholar 

  114. Hatemi G, Melikoglu M, Tunc R, Korkmaz C, Ozturk BT, Mat C, et al. Apremilast for Behcet’s syndrome-A phase 2, placebo-controlled study. N Engl J Med. 2015;372:1510–8.

    Article  CAS  PubMed  Google Scholar 

  115. Mumcu G, Ergun T, Elbir Y, et al. Clinical and immunological effects of azithromycin in Behçet’s disease. J Oral Pathol Med. 2005;34:13–6.

    Article  CAS  PubMed  Google Scholar 

  116. Hamuryudan V, Yurdakul S, Serdaroglu S, Tuzun Y, Rosenkaimer F, Yazici H. Topical alpha interferon in the treatment of oral ulcers in Behçet’s syndrome: a preliminary report. Clin Exp Rheumatol. 1990;8:51–4.

    CAS  PubMed  Google Scholar 

  117. Ergun T, Gurbuz O, Yurdakul S, Hamuryudan V, Bekiroglu N, Yazici H. Topical cyclosporine-A for treatment of oral ulcers of Behçet’s syndrome. Int J Dermatol. 1997;36:720.

    Article  CAS  PubMed  Google Scholar 

  118. Mat C, Tuzun T, Ozsoy Y, Erturk G, Mercan E, Fresko I, et al. Cromolyn gel 4% in the treatment of genital ulcers of Behçet’s disease. In: Bang D, Lee ES, Lee S, editors. Proceeding of the international conference on Behçet’s disease. Seoul: Design Mecca Publishing Co.; 2000. p. 907.

    Google Scholar 

  119. Tasli L, Mat C, De Simone C, Yazici H. Lactobacilli lozenges in the management of oral ulcers of Behçet’s syndrome. Clin Exp Rheumatol. 2006;24:S83–6.

    CAS  PubMed  Google Scholar 

  120. Wilder EG, Frieder J, Sulhan S, Michel P, Cizenski J, Wright JM, Menter MA. Spectrum of orocutaneous disease associations: genodermatoses and inflammatory conditions. J Am Acad Dermatol. 2017;77(5):809–30.

    Article  PubMed  Google Scholar 

  121. Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77:808–18.

    PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Dermatologist, Private Practice, Istanbul, Turkey

    M. Cem Mat

  2. Department of Dermatology, Catholic Kwandong University International St. Mary’s Hospital, Incheon, South Korea

    Dongsik Bang

  3. Department of Dermatology, Ajou University School of Medicine, Suwon, South Korea

    Eun-So Lee

  4. Department of Dermatology, Istanbul University, Cerrahpasa Medical Faculty Hospital, Istanbul, Turkey

    Zekayi Kutlubay

Authors
  1. M. Cem Mat
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  2. Dongsik Bang
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  3. Eun-So Lee
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  4. Zekayi Kutlubay
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Editor information

Editors and Affiliations

  1. School of Medicine, New York University, New York, NY, USA

    Yusuf Yazici

  2. Department of Internal Medicine, Istanbul University, Istanbul, Turkey

    Gulen Hatemi

  3. Department of Rheumatology, Istanbul University, Istanbul, Turkey

    Emire Seyahi

  4. Department of Medicine, Academic Hospital, Istanbul, Turkey

    Hasan Yazici

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Mat, M.C., Bang, D., Lee, ES., Kutlubay, Z. (2020). The Mucocutaneous Manifestations and Pathergy Reaction in Behçet Syndrome. In: Yazici, Y., Hatemi, G., Seyahi, E., Yazici, H. (eds) Behçet Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-24131-5_4

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