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Sections Dermatologic Aspects of Behcet Disease
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DDx
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Treatment
Medication
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References

Medication

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.

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)

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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)

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

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

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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.

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)

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

Class Summary

These agents may be immunomodulatory.

Tetracycline (Sumycin)

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

Class Summary

These agents are used for topical treatment for aphthae.

Amlexanox (Aphthasol)

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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)

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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.

Class Summary

These agents are indicated for autoimmune diseases.

Azathioprine (Imuran)

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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)

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

Tacrolimus (Prograf, Tacrine, FK506)

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Tacrolimus suppresses humoral immunity (T lymphocyte) activity.

Cyclosporine (Sandimmune, Neoral)

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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)

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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)

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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.

Class Summary

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

Thalidomide (Thalomid)

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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)

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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)

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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)

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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.

Behcet H. Uber rezidivierende, aphthose, durchein Virus verursachte Geschwure am Mund, am Auge und anden Genitalien. Dermatol Wochenschr. 1937. 36:1152-7.
Feigenbaum A. Description of Behçet's syndrome in the Hippocratic third book of endemic diseases. Br J Ophthalmol. 1956 Jun. 40(6):355-7. [QxMD MEDLINE Link]. [Full Text].
Mizuki N, Inoko H, Ohno S. Molecular genetics (HLA) of Behcet's disease. Yonsei Med J. 1997 Dec. 38(6):333-49. [QxMD MEDLINE Link].
Elfishawi MM, Elgengehy F, Mossallam G, Elfishawi S, Alfishawy M, Gad A, et al. HLA Class I in Egyptian patients with Behçet's disease: new association with susceptibility, protection, presentation and severity of manifestations. Immunol Invest. 2019 Feb. 48 (2):121-129. [QxMD MEDLINE Link].
Takeuchi M, Kastner DL, Remmers EF. The immunogenetics of Behçet's disease: A comprehensive review. J Autoimmun. 2015 Nov. 64:137-48. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Gül A. Genetics of Behçet's disease: lessons learned from genomewide association studies. Curr Opin Rheumatol. 2014 Jan. 26(1):56-63. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Afkari B, Babaloo Z, Dolati S, Khabazi A, Jadidi-Niaragh F, Talei M, et al. Molecular analysis of interleukin-10 gene polymorphisms in patients with Behçet's disease. Immunol Lett. 2018 Feb. 194:56-61. [QxMD MEDLINE Link].
Hassouna SS, Tayel MY, ElKaffash DM, Abdelhady AM, Elsayed EH. MicroRNA155 Expression in Relation to BDCAF Scored Behçet's Disease in an Egyptian Patients' Sample. Open Rheumatol J. 2018. 12:115-122. [QxMD MEDLINE Link].
Sohn S. Etiopathology of Behcet's disease: herpes simplex virus infection and animal model. Yonsei Med J. 1997 Dec. 38(6):359-64. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Coit P, Mumcu G, Ture-Ozdemir F, Unal AU, Alpar U, Bostanci N, et al. Sequencing of 16S rRNA reveals a distinct salivary microbiome signature in Behçet's disease. Clin Immunol. 2016 Aug. 169:28-35. [QxMD MEDLINE Link].
Consolandi C, Turroni S, Emmi G, Severgnini M, Fiori J, Peano C, et al. Behçet's syndrome patients exhibit specific microbiome signature. Autoimmun Rev. 2015 Apr. 14 (4):269-76. [QxMD MEDLINE Link].
Akkoç N. Update on the epidemiology, risk factors and disease outcomes of Behçet's disease. Best Pract Res Clin Rheumatol. 2018 Apr. 32 (2):261-270. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Kaabachi W, Bouali E, Berraïes A, Dhifallh IB, Hamdi B, Hamzaoui K, et al. Interleukin-26 is overexpressed in Behçet's disease and enhances Th17 related -cytokines. Immunol Lett. 2017 Oct. 190:177-184. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Zhou ZY, Chen SL, Shen N, Lu Y. Cytokines and Behcet's disease. Autoimmun Rev. 2012 Aug. 11(10):699-704. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Usküdar O, Erdem A, Demiroglu H, Dikmenoglu N. Decreased erythrocyte deformability in Behçet's disease. Clin Hemorheol Microcirc. 2005. 33(2):89-94. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Merashli M, Ster IC, Ames PR. Subclinical atherosclerosis in Behcet's disease: A systematic review and meta-analysis. Semin Arthritis Rheum. 2015 Jul 4. [QxMD MEDLINE Link].
Lee YH, Song GG. Association of circulating resistin, leptin, adiponectin and visfatin levels with Behçet disease: a meta-analysis. Clin Exp Dermatol. 2018 Jan 22. [QxMD MEDLINE Link].
Ng WL, Adeeb F, Devlin J, Fraser A. The relationship between Behçet’s disease flare up and menstruation in an Irish cohort. (P123). Clin Exp Rheumatol. 2018. 36 (Suppl. 115):S202.
Muruganandam M, Rolle NA, Sibbitt WL Jr, Cook GB, Emil NS, Fangtham M, et al. Characteristics of Behcet's Disease in the American Southwest. Semin Arthritis Rheum. 2019 Mar 9. [QxMD MEDLINE Link].
Mahr A, Maldini C. [Epidemiology of Behçet's disease]. Rev Med Interne. 2014 Feb. 35(2):81-9. [QxMD MEDLINE Link].
Kim JN, Kwak SG, Choe JY, Kim SK. The prevalence of Behçet's disease in Korea: data from Health Insurance Review and Assessment Service from 2011 to 2015. Clin Exp Rheumatol. 2017 Nov-Dec. 35 Suppl 108 (6):38-42. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [Full Text].
Ishido T, Horita N, Takeuchi M, Kawagoe T, Shibuya E, Yamane T, et al. Clinical manifestations of Behçet's disease depending on sex and age: results from Japanese nationwide registration. Rheumatology (Oxford). 2017 Nov 1. 56 (11):1918-1927. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Na SJ, Kang MJ, Yu DS, Han KD, Lee JH, Park YG, et al. Cancer risk in patients with Behçet disease: A nationwide population-based dynamic cohort study from Korea. J Am Acad Dermatol. 2018 Mar. 78 (3):464-470.e2. [QxMD MEDLINE Link].
Tascilar K, Melikoglu M, Ugurlu S, Sut N, Caglar E, Yazici H. Vascular involvement in Behçet's syndrome: a retrospective analysis of associations and the time course. Rheumatology (Oxford). 2014 Nov. 53 (11):2018-22. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Seyahi E, Caglar E, Ugurlu S, Kantarci F, Hamuryudan V, Sonsuz A, et al. An outcome survey of 43 patients with Budd-Chiari syndrome due to Behçet's syndrome followed up at a single, dedicated center. Semin Arthritis Rheum. 2015 Apr. 44 (5):602-609. [QxMD MEDLINE Link].
Demir S, Sag E, Dedeoglu F, Ozen S. Vasculitis in Systemic Autoinflammatory Diseases. Front Pediatr. 2018. 6:377. [QxMD MEDLINE Link].
Mizushima Y. [Revised diagnostic criteria for Behcet's disease in 1987]. Ryumachi. 1988 Feb. 28(1):66-70. [QxMD MEDLINE Link].
International Study Group for Behcet's Disease. Criteria for diagnosis of Behcet's disease. Lancet. 1990 May 5. 335(8697):1078-80. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Yurdakul S, Yazici H. Behçet's syndrome. Best Pract Res Clin Rheumatol. 2008 Oct. 22(5):793-809. [QxMD MEDLINE Link].
Sharquie KE, Al-Araji A, Hatem A. Oral pathergy test in Behçet's disease. Br J Dermatol. 2002 Jan. 146(1):168-9. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Lee ES, Bang D, Lee S. Dermatologic manifestation of Behçet's disease. Yonsei Med J. 1997 Dec. 38(6):380-9. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Kechida M, Salah S, Kahloun R, Klii R, Hammami S, Khochtali I. Cardiac and vascular complications of Behçet disease in the Tunisian context: clinical characteristics and predictive factors. Adv Rheumatol. 2018 Oct 1. 58 (1):32. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
Mejri M, Said F, Khanfir M, Chelly I, Haouet S, Habib H. [Ulcerations of the extremities: An unusual manifestation of Behçet's disease]. Rev Med Interne. 2019 Feb. 40 (2):117-119. [QxMD MEDLINE Link].
Alpsoy E, Zouboulis CC, Ehrlich GE. Mucocutaneous lesions of Behcet's disease. Yonsei Med J. 2007 Aug 31. 48 (4):573-85. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Seyahi E, Melikoglu M, Yazici H. Clinical features and diagnosis of Behcet's syndrome. Int J Adv Rheumatol. 2007. 5:8.
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Lee YP, Cho CH, Chi HS, et al. Intestinal Behcet's disease. J Korean Surg Soc. 1988. 35:310-5.
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. [QxMD MEDLINE Link].
Kim NH, Yang IH, Kim SM, Bang D. Behcet's arthritis. J Korean Orthop Surg. 1993. 28:1890-7.
Dalvi SR, Yildirim R, Yazici Y. Behcet's Syndrome. Drugs. 2012 Dec 3. 72(17):2223-41. [QxMD MEDLINE Link].
Alonso S, Riveros-Frutos A, Martínez-Morillo M, Grau-Ferrer L, Carrato C, Olivé A. Pseudotumoral Behçet's disease. Reumatol Clin. 2015 Jun 12. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Kanda N, Watanabe S. Regulatory roles of sex hormones in cutaneous biology and immunology. J Dermatol Sci. 2005 Apr. 38(1):1-7. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Saleh Z, Arayssi T. Update on the therapy of Behçet disease. Ther Adv Chronic Dis. 2014 May. 5(3):112-34. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Atmaca L, Boyvat A, Yalçindag FN, Atmaca-Sonmez P, Gurler A. Behçet disease in children. Ocul Immunol Inflamm. 2011 Apr. 19(2):103-7. [QxMD MEDLINE Link].
Chang YS, Yang YH, Chiang BL. Neonatal Behçet's disease without maternal history. Clin Rheumatol. 2011 Dec. 30(12):1641-5. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Baptista B, Marto N, Tavares JG, Horta AB, Cunha E Sá D, Gerardo G, et al. Behçet Syndrome and Crohn's Disease: What Are the Differences?. Eur J Case Rep Intern Med. 2019. 6 (3):001044. [QxMD MEDLINE Link].
Varol A, Seifert O, Anderson CD. The skin pathergy test: innately useful?. Arch Dermatol Res. 2010 Apr. 302 (3):155-68. [QxMD MEDLINE Link].
Pu L, Li R, Xie J, Yang Y, Liu G, Wang Y, et al. Characteristic Echocardiographic Manifestations of Behçet's Disease. Ultrasound Med Biol. 2018 Apr. 44 (4):825-830. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Sfikakis PP. Behcet's disease: a new target for anti-tumour necrosis factor treatment. Ann Rheum Dis. 2002 Nov. 61 Suppl 2:ii51-3. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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.
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Estrach C, Mpofu S, Moots RJ. Behcet's syndrome: response to infliximab after failure of etanercept. Rheumatology (Oxford). 2002 Oct. 41(10):1213-4. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Ozguler Y, Leccese P, Christensen R, et al. Management of major organ involvement of Behçet's syndrome: a systematic review for update of the EULAR recommendations. Rheumatology (Oxford). 2018 Dec 1. 57 (12):2200-2212. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Fekih M, Fennira S, Ghodbane L, Zaouali RM. [Intracardiac thrombosis: unusual complication of Behcet's disease]. Tunis Med. 2004 Aug. 82 (8):785-90. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Chen Y, Shen Y, Ma HF, Cai JF, Hua YQ, Zou J, et al. Infliximab associated with life-threatening lung infection in a patient with Behcet disease with intestinal and hematopoietic system involvement: A case report. Medicine (Baltimore). 2017 Dec. 96 (50):e9202. [QxMD MEDLINE Link].
Hamada K, Takei R, Sakiyama Y, Moriyama H, Hashiguchi A, Takashima H. [A case of chronic progressive neuro-Behçet disease with extensive cerebral atrophy and elevated CSF IL-6 activity treated with infliximab]. Rinsho Shinkeigaku. 2018 Jan 26. 58 (1):30-34. [QxMD MEDLINE Link].
Hisamatsu T, Ueno F, Matsumoto T, Kobayashi K, Koganei K, Kunisaki R, et al. The 2nd edition of consensus statements for the diagnosis and management of intestinal Behçet's disease: indication of anti-TNFα monoclonal antibodies. J Gastroenterol. 2014 Jan. 49 (1):156-62. [QxMD MEDLINE Link].

Media Gallery

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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Author

Amira M Elbendary, MD, MBBCh, MSc Visiting Dermatopathology Fellow, Ackerman Academy of Dermatopathology; Lecturer, Department of Dermatology, Kasr Alainy University Hospitals, Cairo University, Egypt

Amira M Elbendary, MD, MBBCh, MSc is a member of the following medical societies: Bloom’s Syndrome Association, Egyptian Medical Syndicate, International Dermoscopy Society, Medical Dermatology 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 Former 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: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

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: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

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