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Dermatologic Aspects of Behcet Disease Medication

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

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Corticosteroids have been used for all of the various clinical manifestations of Behçet disease. Anti-inflammatory and immunosuppressive agents are used to treat mucocutaneous lesions and arthritis associated with this disease.

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Corticosteroids

Class Summary

These agents modify the body's immune response to diverse stimuli and therefore have anti-inflammatory properties. In addition, they cause profound and varied metabolic effects. Corticosteroids are immunosuppressive and affect the replication, movement, and activity of virtually all cells involved with inflammation.

Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

 

Prednisone is an immunosuppressant used for the treatment of autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisone stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

Hydrocortisone (Solu-Cortef)

 

Hydrocortisone decreases inflammation by suppressing migration of PMN leukocytes and reversing increased capillary permeability.

Methylprednisolone (Medrol, Medrol Dosepak, Depo Medrol, Solu-Medrol)

 

Methylprednisolone exerts anti-inflammatory effects by suppressing the migration of polymorphonuclear leukocytes and reversing increased capillary permeability. Methylprednisolone sodium succinate (Solu-Medrol) is highly soluble and has a rapid effect by intramuscular and intravenous routes. Depo Medrol is available as intramuscular, intra-articular, and intralesional injections.

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Nonsteroidal anti-inflammatory drugs

Class Summary

Although most NSAIDs are used primarily for their anti-inflammatory effects, they are effective analgesics and used to treat mild to moderate pain.

Ibuprofen (Ibuprin, Advil, Motrin)

 

Ibuprofen is the drug of choice for mild to moderate pain. It inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.

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Antibiotics

Class Summary

These agents may be immunomodulatory.

Tetracycline (Sumycin)

 

Tetracycline inhibits bacterial protein synthesis by binding with 30S and, possibly, 50S ribosomal subunit(s). It has anti-inflammatory activity.

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Antiulcer agents

Class Summary

These agents are used for topical treatment for aphthae.

Amlexanox (Aphthasol)

 

Amlexanox is a topical mucosal anti-inflammatory agent paste at 5% concentration. The exact mechanism of action remains unknown. Begin treatment as soon as the patient notices symptoms.

Sucralfate (Carafate, Sulcrate)

 

Sucralfate forms a viscous adhesive substance that protects the GI lining against pepsin, peptic acid, and bile salts. Use it for short-term management of ulcers.

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Immunosuppressive agents

Class Summary

These agents are indicated for autoimmune diseases.

Azathioprine (Imuran)

 

Azathioprine antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. It may decrease proliferation of immune cells, which results in lower autoimmune activity.

Chlorambucil (Leukeran)

 

Chlorambucil alkylates and cross-links strands of DNA, inhibiting DNA replication and RNA transcription.

Tacrolimus (Prograf, Tacrine, FK506)

 

Tacrolimus suppresses humoral immunity (T lymphocyte) activity.

Cyclosporine (Sandimmune, Neoral)

 

Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft versus host disease for a variety of organs. For children and adults, base dosing on ideal body weight.

Cyclophosphamide (Cytoxan)

 

Cyclophosphamide is an alkylating agent that prevents cell division by cross-linking DNA strands and thus interfering with DNA replication. It has potent immunosuppressive activity. Cyclophosphamide is a prodrug that must be metabolized by the liver into active metabolites.

Cyclophosphamide has serious adverse effects, including bladder cancer, hemorrhagic cystitis, acute myeloid leukemia, and permanent infertility.

Closely monitor the CBC count with differential and platelets, BUN, and serum creatinine for signs and symptoms of hemorrhagic cystitis; also monitor liver function test results.

Colchicine (Colcrys, Mitigare)

 

Colchicine prevents activation, degranulation, and migration of neutrophils. It is contraindicated in patients with renal and hepatic impairment. Aplastic anemia and myelosuppression (eg, leukopenia, granulocytopenia, thrombocytopenia, pancytopenia) have been reported in patients receiving therapeutic doses.

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Immunomodulators

Class Summary

Immunomodulators are anti-inflammatory agents that modulate the immune system through a variety of mechanisms.

Thalidomide (Thalomid)

 

Thalidomide is an immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.

Infliximab (Remicade)

 

Infliximab neutralizes cytokine TNF-alpha and inhibits its binding to the TNF-alpha receptor. Mix in 250 mL normal saline for infusion over 2 hours. It must be used with a low protein-binding filter (≥ 1.2 µm). It has been used off label for treating BD.

Etanercept (Enbrel)

 

Etanercept is a soluble p75 TNF receptor fusion protein (sTNFR-Ig). It inhibits TNF binding to cell surface receptors, which, in turn, decreases inflammatory and immune responses. It is off-label use for Behçet disease.

Adalimumab (Humira)

 

Adalimumab is a recombinant human anti-TNF-α IgG1 monoclonal antibody. It specifically binds to TNF-α and blocks its interaction with p55 and p75 cell surface TNF receptors and, accordingly, decreases the inflammatory and immune response. It is off-label use for Behçet disease.

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

Acknowledgements

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.

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. 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].

 
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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.
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.
 
 
 
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