Ulcerative lesions in Behcet’s disease (BD) are regarded as important manifestation for diagnosis. Various kinds of ulcerative lesions appear in patients with BD. They present as orogenital ulcers, necrotizing vasculitis and pyoderma gengrenosum. Gastrointestinal system involvement (Gis) in Beh？et’s disease affects all areas from the esophagus to the anus. Most authors believe that the Gis manifestations of Beh？et’s disease should be confined to aphthous ulcers, which can occur throughout the Gis tract. All patients with oro-genital and Gis ulcerations should be fully investigated to establish a definitive diagnosis and eliminate the possibility of an underlying BD. 1. Introduction BD was first defined by Beh？et, a Turkish Professor of Dermatology, in 1937 as a triad of recurrent aphthous stomatitis, genital aphthae, and relapsing uveitis [1]. During the ensuing 65 years multiple systemic associations of the disease including articular, vascular, gastrointestinal, cardiopulmonary and neurologic involvement have become increasingly apparent [2–4]. Although the etiology and pathogenesis is not clearly defined, genetic predisposition, infections and immunological dysfunctions have been implicated [5]. BD has been reported worldwide, but has a distinct geographic distribution, with highest prevalences in countries along the ancient silk route. Although much has been learned during recent years on the pathogenesis and treatment of the disease, it is still an important cause of morbidity and mortality in areas where it is prevalent [3]. Young individuals are most commonly affected. Male to female ratio is usually 1？:？1. The gender predominance is different according to the prevalent countries. So M？:？F ratio 1？:？1 seems to be not always correct [4]. Ocular and central nervous system involvement are the basic prognostic factors in BD. Cardiovascular, pulmonary, and gastrointestinal system involvements are the major causes of mortality. In different series, high prevalence of ocular, nervous system, pulmonary system involvement, large vessel thrombosis, thrombophlebitis and patergy positivity has been found in male patients, and in view of these data a more severe course in male patients can be expected. Higher incidence of severe clinical course and systemic involvement is observed when early onset of the disease is present [2–4]. 2. Ulcerative Clinical Manifestations Ulcerative lesions in BD are regarded as important manifestation for diagnosis. Various kinds of ulcerative mucocutaneous and Gis lesions appear in patients with BD. 2.1. Mucocutaneous Ulcerative
References
[1]
H. Beh？et, “Uber rezidivierende aphthouse durch ein virus verursachte Geschwuere am Mund, am Auge und an den Genitalien,” Dermatologische Wochenschrift, vol. 105, pp. 1152–1157, 1937.
[2]
A. Gurler, A. Boyvat, and U. Tursen, “Clinical manifestations of Beh？et's disease: an analysis of 2147 patients,” Yonsei Medical Journal, vol. 38, no. 6, pp. 423–427, 1997.
[3]
G. Azizlerli, A. A. Kose, R. Sarica, et al., “Prevalence of Behcet’s disease in Istanbul, Turkey,” International Journal of Dermatology, vol. 42, pp. 803–806, 2003.
[4]
U. Tursen, A. Gurler, and A. Boyvat, “Evaluation of clinical findings according to sex in 2313 Turkish patients with Beh？et's disease,” International Journal of Dermatology, vol. 42, no. 5, pp. 346–351, 2003.
[5]
E. Alpsoy, C. Zouboulis, and G. E. Ehrlich, “Mucocutaneous lesions of Beh？et's disease,” Yonsei Medical Journal, vol. 48, no. 4, pp. 573–585, 2007.
[6]
P. Scheid, A. Bohadana, and Y. Martinet, “Nicotine patches for aphthous ulcers due to Behcet's syndrome,” New England Journal of Medicine, vol. 343, no. 24, pp. 1816–1817, 2000.
[7]
M. C. Mat, N. Goksugur, B. Engin, S. Yurdakul, and H. Yazici, “The frequency of scarring after genital ulcers in Beh？et's syndrome: a prospective study,” International Journal of Dermatology, vol. 45, no. 5, pp. 554–556, 2006.
[8]
S. Rashtak and M. Pittelkow, “Skin involvement in systemic autoimmune diseases,” Current Directions in Autoimmunity, vol. 10, pp. 344–358, 2008.
[9]
O. Gündüz, “Histopathological evaluation of beh？et's disease and identification of new skin lesions,” Pathology Research International, vol. 2012, Article ID 209316, 7 pages, 2012.
[10]
H. Yazici, I. Fresko, V. Hamuryudan et al., “Beh？et's syndrome: the cerrahpasa experience,” Advances in Experimental Medicine and Biology, vol. 455, pp. 135–140, 1999.
[11]
J. W. Kim, J. H. Park, D. Lee, S. W. Hwang, and S. W. Park, “Vegetative pyoderma gangrenosum in Beh？et's disease,” Acta Dermato-Venereologica, vol. 87, no. 4, pp. 365–367, 2007.
[12]
M. Melikoglu, E. Kural-Seyahi, K. Tascilar, and H. Yazici, “The unique features of vasculitis in Beh？et's syndrome,” Clinical Reviews in Allergy and Immunology, vol. 35, no. 1-2, pp. 40–46, 2008.
[13]
O. S. Vasculopathy, “Behcet’s syndrome, and familial Mediterranean fever,” Current Opinion in Rheumatology, vol. 11, pp. 393–398, 1999.
[14]
I. Krause and A. Weinberger, “Behcet’s disease,” Current Opinion in Rheumatology, vol. 20, pp. 82–87, 2008.
[15]
U. Türsen, B. Ulubas, T. Irfan Kaya, H. Pekdemir, and G. Ikizo？lu, “Cardiac complications in Beh？et's disease,” Clinical and Experimental Dermatology, vol. 27, no. 8, pp. 651–653, 2002.
[16]
G. R. Plotkin, B. R. Patel, and V. N. Shah, “Beh？et's syndrome complicated by cutaneous leukocytoclastic vasculitis. Response to prednisone and chlorambucil,” Archives of Internal Medicine, vol. 145, no. 10, pp. 1913–1915, 1985.
[17]
A. Vikas, S. Atul, R. Singh, L. Sarbmeet, and H. Mohan, “Beh？et's disease with relapsing cutaneous polyarteritis-nodosa-like lesions, responsive to oral cyclosporine therapy,” Dermatology Online Journal, vol. 9, no. 5, p. 9, 2003.
[18]
S. E. Marshall, “Beh？et's disease,” Best Practice and Research: Clinical Rheumatology, vol. 18, no. 3, pp. 291–311, 2004.
[19]
C. Evereklioglu, “Current concepts in the etiology and treatment of Beh？et disease,” Survey of Ophthalmology, vol. 50, no. 4, pp. 297–350, 2005.
[20]
J. Andrews and D. O. Haskard, “Current management options in beh？et's disease,” Minerva Medica, vol. 93, no. 5, pp. 335–345, 2002.
[21]
M. C. Pickering and D. O. Haskard, “Beh？et's syndrome,” Journal of the Royal College of Physicians of London, vol. 34, no. 2, pp. 169–177, 2000.
[22]
A. Kokturk, “Clinical and pathological manifestations with differential diagnosis in Beh？et's disease,” Pathology Research International, vol. 2012, Article ID 690390, 9 pages, 2012.
[23]
I. Krause, Y. Molad, M. Mitrani, and A. Weinberger, “Pathergy reaction in Behcet's disease: lack of correlation with mucocutaneous manifestations and systemic disease expression,” Clinical and Experimental Rheumatology, vol. 18, no. 1, pp. 71–74, 2000.
[24]
ü. Gül and M. G？nül, “Oral and genital pathergy in Beh？et's disease,” Dermatology, vol. 215, no. 1, pp. 80–81, 2007.
[25]
J. Andrews and D. O. Haskard, “Current management options in beh？et's disease,” Minerva Medica, vol. 93, no. 5, pp. 335–345, 2002.
[26]
E. Alpsoy, L. Donmez, M. Onder et al., “Clinical features and natural course of Beh？et's disease in 661 cases: a multicentre study,” British Journal of Dermatology, vol. 157, no. 5, pp. 901–906, 2007.
[27]
E. Alpsoy and A. Akman, “Beh？et's disease: an algorithmic approach to its treatment,” Archives of Dermatological Research, vol. 301, no. 10, pp. 693–702, 2009.
[28]
A. Khandwala, R. G. Van Inwegen, and M. C. Alfano, “5% amlexanox oral paste, a new treatment for recurrent minor aphthous ulcers: I. Clinical demonstration of acceleration of healing and resolution of pain,” Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics, vol. 83, no. 2, pp. 222–230, 1997.
[29]
C. Tanaka, T. Matsuda, Y. Yukinari, H. Yamada, Y. Ichikawa, T. Sakane, et al., “The beneficial effect of rebamipide on recurrent oral aphthous ulcers in Behcet’s disease,” in Behcet’s Disease, M. Hamza, Ed., pp. 477–480, Pub Adhoua, Tunis, Tunisia, 1997.
[30]
E. Alpsoy, H. Er, C. Durusoy, and E. Yilmaz, “The use of sucralfate suspension in the treatment of oral and genital ulceration of Behcet disease: a randomized, placebo-controlled, double-blind study,” Archives of Dermatology, vol. 135, no. 5, pp. 529–532, 1999.
[31]
M. A. G. Edres, C. Scully, and M. Gelbier, “Use of proprietary agents to relieve recurrent aphthous stomatitis,” British Dental Journal, vol. 182, no. 4, pp. 144–146, 1997.
[32]
N. Alli, G. Karakayali, I. Kahraman, and F. Artuz, “Local intralesional therapy with rhGM-CSF for a large genital ulcer in Behcet's disease,” British Journal of Dermatology, vol. 136, no. 4, pp. 639–640, 1997.
[33]
I. Kotter, H. Durk, J. Saal, G. Fierlbeck, U. Pleyer, and M. Zierhut, “Therapy of Behcet’s disease,” German Journal of Ophthalmology, vol. 5, pp. 92–97, 1996.
[34]
K. E. Sharquie, R. A. Najim, W. S. Al-Dori, and R. K. Al-Hayani, “Oral zinc sulfate in the treatment of Behcet's disease: a double blind cross-over study,” Journal of Dermatology, vol. 33, no. 8, pp. 541–546, 2006.
[35]
V. G. Kaklamani and P. G. Kaklamanis, “Treatment of beh？et's disease—an update,” Seminars in Arthritis and Rheumatism, vol. 30, no. 5, pp. 299–312, 2001.
[36]
H. Yazici, S. Yurdakul, and V. Hamuryudan, “Beh？et disease,” Current Opinion in Rheumatology, vol. 13, no. 1, pp. 18–22, 2001.
[37]
J. M. M. Gardner-Medwin, N. J. Smith, and R. J. Powell, “Clinical experience with thalidomide in the management of severe oral and genital ulceration in conditions such as Behcet's disease: use of neurophysiological studies to detect thalidomide neuropathy,” Annals of the Rheumatic Diseases, vol. 53, no. 12, pp. 828–832, 1994.
[38]
P. De Merieux, L. E. Spitler, and H. E. Paulus, “Treatment of Behcet's syndrome with levamisole,” Arthritis and Rheumatism, vol. 24, no. 1, pp. 64–70, 1981.
[39]
I. Fresko, S. Yurdakul, V. Hamuryudan et al., “The management of Behcet's syndrome,” Annales de Medecine Interne, vol. 150, no. 7, pp. 576–581, 1999.
[40]
E. Aktulga, M. Altac, and A. Muftuoglu, “A double blind study of colchicine in Behcet's disease,” Haematologica, vol. 65, no. 3, pp. 399–402, 1980.
[41]
S. Yurdakul, C. Mat, Y. Tuzun, et al., “A double-blind trial of colchicine in Behcet’s syndrome,” Arthritis and Rheumatism, vol. 44, pp. 2686–2692, 2001.
[42]
S. Okten, “Penicillin in treatment of Behcet’s disease,” in Behcet’s Disease: Basic and Clinical Aspects, J. D. O’Duffy and E. Kokmen, Eds., pp. 645–648, Marcel Dekker, New York, NY, USA, 1991.
[43]
I. C. Haznedaroglu, H. Demiroglu, O. I. Ozcebe, O. Ozdemir, and S. V. Dundar, “Benzathine-penicillin in the prophylaxis and treatment of Behcet’s disease,” in Proceedings of the 7th Mediterranean Congress of Rheumatology, K. A. Boki, A. A. Drosos, H. M. Moutsopoulos, A. G. Tzioufas, and P. G. Vlachoyiannopoulos, Eds., pp. 185–188, Monduzzi Editore, Athens, Greece, 1994.
[44]
M. ？algüneri, I. Ertenli, S. Kiraz, M. Erman, and I. ？elik, “Effect of prophylactic benzathine penicillin on mucocutaneous symptoms of Beh？et's disease,” Dermatology, vol. 192, no. 2, pp. 125–128, 1996.
[45]
N. Oyama, M. Inoue, T. Matsui, Y. Nihei, A. Nishibu, and F. Kaneko, “Minocycline effects on the clinical symptoms in correlation with cytokines produced by peripheral blood mononuclear cells stimulated with streptococcal antigens in Behcet’s disease,” in Behcet’s Disease, M. Hamza, Ed., pp. 481–486, Pub Adhoua, Tunis, Tunisia, 1997.
[46]
T. Sakane, M. Takeno, N. Suzuki, and G. Inaba, “Behcet's disease,” New England Journal of Medicine, vol. 341, no. 17, pp. 1284–1291, 1999.
[47]
E. Alpsoy, C. Durusoy, E. Yilmaz et al., “Interferon alfa-2a in the treatment of Beh？et disease: a randomized placebo-controlled and double-blind study,” Archives of Dermatology, vol. 138, no. 4, pp. 467–471, 2002.
[48]
H. Yazici, H. Pazarli, C. G. Barnes et al., “A controlled trial of azathioprine in Behcet's syndrome,” New England Journal of Medicine, vol. 322, no. 5, pp. 281–285, 1990.
[49]
D. BenEzra, E. Cohen, T. Chajek et al., “Evaluation of conventional therapy versus cyclosporine A in Behcet's syndrome,” Transplantation Proceedings, vol. 20, no. 3, pp. 136–143, 1988.
[50]
J. L. Jorizzo, W. L. White, C. M. Wise, M. D. Zanolli, and E. F. Sherertz, “Low-dose weekly methotrexate for unusual neutrophilic vascular reactions: cutaneous polyarteritis nodosa and Behcet's disease,” Journal of the American Academy of Dermatology, vol. 24, no. 6, pp. 973–978, 1991.
[51]
J. L. Jorizzo, F. C. Schmalstieg, and A. R. Solomon, “Thalidomide effects in Behcet's syndrome and pustular vasculitis,” Archives of Internal Medicine, vol. 146, no. 5, pp. 878–881, 1986.
[52]
T. Saylan and I. Saltik, “Thalidomide in the treatment of Behcet's syndrome,” Archives of Dermatology, vol. 118, no. 8, p. 536, 1982.
[53]
A. M. Denman, E. Graham, L. Howe, E. Y. Denman, and S. Lightman, “Low dose thalidomide treatment of Behcet’s syndrome,” in Behcet’s Disease, B. Wechsler and P. Godeau, Eds., International Congress Series, pp. 649–653, Excerpta Medica, Amsterdam, The Netherlands, 1992.
[54]
P. P. Sfikakis, N. Markomichelakis, E. Alpsoy et al., “Anti-TNF therapy in the management of Beh？et's disease—review and basis for recommendations,” Rheumatology, vol. 46, no. 5, pp. 736–741, 2007.
[55]
M. Melikoglu, I. Fresko, C. Mat et al., “Short-term trial of etanercept in Beh？et's disease: a double blind, placebo controlled study,” Journal of Rheumatology, vol. 32, no. 1, pp. 98–105, 2005.
[56]
L. Tasli, C. Mat, C. De Simone, and H. Yazici, “Lactobacilli lozenges in the management of oral ulcers of Beh？et's syndrome,” Clinical and Experimental Rheumatology, vol. 24, pp. S83–S86, 2006.
[57]
H. Larsson, “Treatment of severe colitis in Behcet's syndrome with thalidomide (CG-217),” Journal of Internal Medicine, vol. 228, no. 4, pp. 405–407, 1990.
