Skip to main content
SpringerLink
Log in

Cutaneous Manifestations of Inflammatory Bowel Disease: A Basic Overview

  • Review Article
  • Published:
American Journal of Clinical Dermatology Aims and scope Submit manuscript

Abstract

Inflammatory bowel disease (IBD) is a chronic inflammatory condition of the gastrointestinal (GI) tract that is subdivided into Crohn’s disease (CD) and ulcerative colitis (UC). CD is characterized by involvement of the entire GI tract, while UC mainly affects the distal GI tract. Moreover, both CD and UC can present with extraintestinal manifestations (EIMs) of the disease affecting multiple organ systems including the hepatobiliary tract, kidney, bones, eyes, joints, and skin. These complications can cause significant morbidity and negatively impact the quality of life for IBD patients. Although the pathogenesis of EIMs is not clearly elucidated, it is postulated that the diseased GI mucosa similarly stimulates excess immune responses at the extraintestinal sites. Cutaneous EIMs occur in up to 15% of patients with IBD, often predating their IBD diagnosis. They are categorized into (1) specific, (2) reactive, (3) associated, and (4) treatment-induced. Here, we review the epidemiological, clinical, diagnostic, and histologic features of the most commonly described cutaneous EIMs of IBD along with their respective treatment options.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. McDowell CFU, Haseeb M. Inflammatory Bowel Disease. [Updated 2021 Aug 31]. In: StatPearls [Internet]. Treasure Island: StatPearls Publishing, https://www.ncbi.nlm.nih.gov/books/NBK470312/.

  2. Ananthakrishnan AN. Epidemiology and risk factors for IBD. Nat Rev Gastroenterol Hepatol. 2015;12(4):205–17.

    Article  PubMed  Google Scholar 

  3. Windsor JW, Kaplan GG. Evolving epidemiology of IBD. Curr Gastroenterol Rep. 2019;21(8):40.

    Article  PubMed  Google Scholar 

  4. Aniwan S, Harmsen WS, Tremaine WJ, Loftus EV. Incidence of inflammatory bowel disease by race and ethnicity in a population-based inception cohort from 1970 through 2010. Therap Adv Gastroenterol. 2019;12:1756284819827692.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Sonnenberg A, McCarty DJ, Jacobsen SJ. Geographic variation of inflammatory bowel disease within the United States. Gastroenterology. 1991;100(1):143–9.

    Article  CAS  PubMed  Google Scholar 

  6. Calkins BM, Lilienfeld AM, Garland CF, Mendeloff AI. Trends in incidence rates of ulcerative colitis and Crohn’s disease. Dig Dis Sci. 1984;29(10):913–20.

    Article  CAS  PubMed  Google Scholar 

  7. Roth MP, Petersen GM, McElree C, Feldman E, Rotter JI. Geographic origins of Jewish patients with inflammatory bowel disease. Gastroenterology. 1989;97(4):900–4.

    Article  CAS  PubMed  Google Scholar 

  8. StatPearls. 2021.

  9. van Wijk F, Cheroutre H. Mucosal T cells in gut homeostasis and inflammation. Expert Rev Clin Immunol. 2010;6(4):559–66.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Xavier RJ, Podolsky DK. Unravelling the pathogenesis of inflammatory bowel disease. Nature. 2007;448(7152):427–34.

    Article  CAS  PubMed  Google Scholar 

  11. Das KM, Vecchi M, Sakamaki S. A shared and unique epitope(s) on human colon, skin, and biliary epithelium detected by a monoclonal antibody. Gastroenterology. 1990;98(2):464–9.

    Article  CAS  PubMed  Google Scholar 

  12. Vavricka SR, Schoepfer A, Scharl M, Lakatos PL, Navarini A, Rogler G. Extraintestinal manifestations of inflammatory bowel disease. Inflamm Bowel Dis. 2015;21(8):1982–92.

    Article  PubMed  Google Scholar 

  13. Levine JS, Burakoff R. Extraintestinal manifestations of inflammatory bowel disease. Gastroenterol Hepatol (NY). 2011;7(4):235–41.

    Google Scholar 

  14. Vavricka SR, Rogler G, Gantenbein C, Spoerri M, Prinz Vavricka M, Navarini AA, et al. Chronological order of appearance of extraintestinal manifestations relative to the time of IBD diagnosis in the Swiss inflammatory bowel disease cohort. Inflamm Bowel Dis. 2015;21(8):1794–800.

    Article  PubMed  Google Scholar 

  15. Huang BL, Chandra S, Shih DQ. Skin manifestations of inflammatory bowel disease. Front Physiol. 2012;3:13.

    PubMed  PubMed Central  Google Scholar 

  16. Panackel C, John J, Krishnadas D, Vinayakumar KR. Metastatic Crohn’s disease of external genitalia. Indian J Dermatol. 2008;53(3):146–8.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Lauritano D, Boccalari E, Di Stasio D, Della Vella F, Carinci F, Lucchese A, et al. Prevalence of oral lesions and correlation with intestinal symptoms of inflammatory bowel disease: a systematic review. Diagnostics (Basel). 2019;9(3):77.

    Article  PubMed Central  Google Scholar 

  18. Laube R, Liu K, Schifter M, Yang JL, Suen MK, Leong RW. Oral and upper gastrointestinal Crohn’s disease. J Gastroenterol Hepatol. 2018;33(2):355–64.

    Article  PubMed  Google Scholar 

  19. Grave B, McCullough M, Wiesenfeld D. Orofacial granulomatosis—a 20-year review. Oral Dis. 2009;15(1):46–51.

    Article  CAS  PubMed  Google Scholar 

  20. Lazzerini M, Bramuzzo M, Ventura A. Association between orofacial granulomatosis and Crohn’s disease in children: systematic review. World J Gastroenterol. 2014;20(23):7497–504.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Padmavathi B, Sharma S, Astekar M, Rajan Y, Sowmya G. Oral Crohn’s disease. J Oral Maxillofac Pathol. 2014;18(Suppl 1):S139–42.

    PubMed  PubMed Central  Google Scholar 

  22. Chi AC, Neville BW, Krayer JW, Gonsalves WC. Oral manifestations of systemic disease. Am Fam Physician. 2010;82(11):1381–8.

    PubMed  Google Scholar 

  23. Lankarani KB, Sivandzadeh GR, Hassanpour S. Oral manifestation in inflammatory bowel disease: a review. World J Gastroenterol. 2013;19(46):8571–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Zbar AP, Ben-Horin S, Beer-Gabel M, Eliakim R. Oral Crohn’s disease: is it a separable disease from orofacial granulomatosis? A review. J Crohns Colitis. 2012;6(2):135–42.

    Article  PubMed  Google Scholar 

  25. Kim SK, Lee ES. Orofacial granulomatosis associated with Crohn’s disease. Ann Dermatol. 2010;22(2):203–5.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Rice SA, Woo PN, El-Omar E, Keenan RA, Ormerod AD. Topical tacrolimus 0.1% ointment for treatment of cutaneous Crohn’s disease. BMC Res Notes. 2013;6:19.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Dupuy A, Cosnes J, Revuz J, Delchier JC, Gendre JP, Cosnes A. Oral Crohn disease: clinical characteristics and long-term follow-up of 9 cases. Arch Dermatol. 1999;135(4):439–42.

    Article  CAS  PubMed  Google Scholar 

  28. Sakuntabhai A, MacLeod RI, Lawrence CM. Intralesional steroid injection after nerve block anesthesia in the treatment of orofacial granulomatosis. Arch Dermatol. 1993;129(4):477–80.

    Article  CAS  PubMed  Google Scholar 

  29. Fedele S, Fung PP, Bamashmous N, Petrie A, Porter S. Long-term effectiveness of intralesional triamcinolone acetonide therapy in orofacial granulomatosis: an observational cohort study. Br J Dermatol. 2014;170(4):794–801.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Campbell HE, Escudier MP, Patel P, Challacombe SJ, Sanderson JD, Lomer MC. Review article: cinnamon- and benzoate-free diet as a primary treatment for orofacial granulomatosis. Aliment Pharmacol Ther. 2011;34(7):687–701.

    Article  CAS  PubMed  Google Scholar 

  31. Espinoza I, Navarrete J, Benedetto J, Borzutzky A, Roessler P, Ortega-Pinto A. Orofacial granulomatosis and diet therapy: a review of the literature and two clinical cases. An Bras Dermatol. 2018;93(1):80–5.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Marzano AV, Borghi A, Stadnicki A, Crosti C, Cugno M. Cutaneous manifestations in patients with inflammatory bowel diseases: pathophysiology, clinical features, and therapy. Inflamm Bowel Dis. 2014;20(1):213–27.

    Article  PubMed  Google Scholar 

  33. Quezada S, Turner PL, Alexiev B, Daly B, Cross R. Severe refractory orofacial Crohn’s disease: report of a case. Dig Dis Sci. 2009;54(10):2290–5.

    Article  PubMed  Google Scholar 

  34. Williams DR, Coller JA, Corman ML, Nugent FW, Veidenheimer MC. Anal complications in Crohn’s disease. Dis Colon Rectum. 1981;24(1):22–4.

    Article  CAS  PubMed  Google Scholar 

  35. Sangwan YP, Schoetz DJ, Murray JJ, Roberts PL, Coller JA. Perianal Crohn’s disease. Results of local surgical treatment. Dis Colon Rectum. 1996;39(5):529–35.

    Article  CAS  PubMed  Google Scholar 

  36. Singh B, McC Mortensen NJ, Jewell DP, George B. Perianal Crohn’s disease. Br J Surg. 2004;91(7):801–14.

    Article  CAS  PubMed  Google Scholar 

  37. Safar B, Sands D. Perianal Crohn’s disease. Clin Colon Rectal Surg. 2007;20(4):282–93.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Danese S, Semeraro S, Papa A, Roberto I, Scaldaferri F, Fedeli G, et al. Extraintestinal manifestations in inflammatory bowel disease. World J Gastroenterol. 2005;11(46):7227–36.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Schwartz DA, Tagarro I, Carmen Díez M, Sandborn WJ. Prevalence of fistulizing Crohn’s disease in the United States: estimate from a systematic literature review attempt and population-based database analysis. Inflamm Bowel Dis. 2019;25(11):1773–9.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Sandborn WJ, Fazio VW, Feagan BG, Hanauer SB, Committee AGACP. AGA technical review on perianal Crohn’s disease. Gastroenterology. 2003;125(5):1508–30.

    Article  PubMed  Google Scholar 

  41. Egal A, London J, Lidove O, Atienza P, Etienney I. Anorectal manifestations in systemic diseases. Rev Med Interne. 2019;40(11):729–32.

    Article  CAS  PubMed  Google Scholar 

  42. Panés J, Rimola J. Perianal fistulizing Crohn’s disease: pathogenesis, diagnosis and therapy. Nat Rev Gastroenterol Hepatol. 2017;14(11):652–64.

    Article  PubMed  Google Scholar 

  43. Bataille F, Klebl F, Rümmele P, Schroeder J, Farkas S, Wild PJ, et al. Morphological characterisation of Crohn’s disease fistulae. Gut. 2004;53(9):1314–21.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. de Zoeten EF, Pasternak BA, Mattei P, Kramer RE, Kader HA. Diagnosis and treatment of perianal Crohn disease: NASPGHAN clinical report and consensus statement. J Pediatr Gastroenterol Nutr. 2013;57(3):401–12.

    Article  PubMed  Google Scholar 

  45. Ickrath F, Stoevesandt J, Schulmeyer L, Glatzel C, Goebeler M, Kerstan A. Metastatic Crohn’s disease: an underestimated entity. J Dtsch Dermatol Ges. 2021;19(7):973–82.

    PubMed  Google Scholar 

  46. Shields BE, Richardson C, Arkin L, Kornik R. Vulvar Crohn disease: diagnostic challenges and approach to therapy. Int J Womens Dermatol. 2020;6(5):390–4.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Abboud ME, Frasure SE. Vulvar inflammation as a manifestation of Crohn’s disease. World J Emerg Med. 2017;8(4):305–7.

    Article  PubMed  PubMed Central  Google Scholar 

  48. Bhoyrul B, Lyon C. Crohn’s disease of the vulva: a prospective study. J Gastroenterol Hepatol. 2018;33(12):1969–74.

    Article  PubMed  Google Scholar 

  49. Barret M, de Parades V, Battistella M, Sokol H, Lemarchand N, Marteau P. Crohn’s disease of the vulva. J Crohns Colitis. 2014;8(7):563–70.

    Article  PubMed  Google Scholar 

  50. Batra J, Goraya SK, Grewal S, Singh A. Metastatic Crohn’s disease of the vulva: a rare presentation. Indian Dermatol Online J. 2020;11(3):416–8.

    Article  PubMed  PubMed Central  Google Scholar 

  51. Granese R, Calagna G, Morabito G, Carriero C, Perino A, Tonni G, et al. Vulvar involvement in pediatric Crohn’s disease: a systematic review. Arch Gynecol Obstet. 2018;297(1):3–11.

    Article  PubMed  Google Scholar 

  52. Loftus EV. Clinical epidemiology of inflammatory bowel disease: incidence, prevalence, and environmental influences. Gastroenterology. 2004;126(6):1504–17.

    Article  PubMed  Google Scholar 

  53. Siroy A, Wasman J. Metastatic Crohn disease: a rare cutaneous entity. Arch Pathol Lab Med. 2012;136(3):329–32.

    Article  PubMed  Google Scholar 

  54. Burgdorf W. Cutaneous manifestations of Crohn’s disease. J Am Acad Dermatol. 1981;5(6):689–95.

    Article  CAS  PubMed  Google Scholar 

  55. Aberumand B, Howard J. Metastatic Crohn’s disease: an approach to an uncommon but important cutaneous disorder. Biomed Res Int. 2017;2017:8192150.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Laftah Z, Bailey C, Zaheri S, Setterfield J, Fuller LC, Lewis F. Vulval Crohn’s disease: a clinical study of 22 patients. J Crohns Colitis. 2015;9(4):318–25.

    Article  PubMed  Google Scholar 

  57. Chakiri R, Baybay H, Hatimi AE, Gallouj S, Harmouch T, Mernissi FZ. Clinical and histological patterns and treatment of pyoderma gangrenosum. Pan Afr Med J. 2020;36:59.

    Article  PubMed  PubMed Central  Google Scholar 

  58. Keyal U, Liu Y, Bhatta AK. Dermatologic manifestations of inflammatory bowel disease: a review. Discov Med. 2018;25(139):225–33.

    PubMed  Google Scholar 

  59. States V, O’Brien S, Rai JP, Roberts HL, Paas M, Feagins K, et al. Pyoderma gangrenosum in inflammatory bowel disease: a systematic review and meta-analysis. Dig Dis Sci. 2020;65(9):2675–85.

    Article  PubMed  Google Scholar 

  60. Rothfuss KS, Stange EF, Herrlinger KR. Extraintestinal manifestations and complications in inflammatory bowel diseases. World J Gastroenterol. 2006;12(30):4819–31.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. Xia FD, Liu K, Lockwood S, Butler D, Tsiaras WG, Joyce C, et al. Risk of developing pyoderma gangrenosum after procedures in patients with a known history of pyoderma gangrenosum—a retrospective analysis. J Am Acad Dermatol. 2018;78(2):310-4.e1.

    Article  PubMed  Google Scholar 

  62. Powell FC, Su WP, Perry HO. Pyoderma gangrenosum: classification and management. J Am Acad Dermatol. 1996;34(3):395–409 (quiz 10–12).

    Article  CAS  PubMed  Google Scholar 

  63. Brooklyn T, Dunnill G, Probert C. Diagnosis and treatment of pyoderma gangrenosum. BMJ. 2006;333(7560):181–4.

    Article  PubMed  PubMed Central  Google Scholar 

  64. Jockenhöfer F, Wollina U, Salva KA, Benson S, Dissemond J. The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum. Br J Dermatol. 2019;180(3):615–20.

    Article  PubMed  Google Scholar 

  65. Harbord M, Annese V, Vavricka SR, Allez M, Barreiro-de Acosta M, Boberg KM, et al. The first European evidence-based consensus on extra-intestinal manifestations in inflammatory bowel disease. J Crohns Colitis. 2016;10(3):239–54.

    Article  PubMed  Google Scholar 

  66. Greuter T, Navarini A, Vavricka SR. Skin manifestations of inflammatory bowel disease. Clin Rev Allergy Immunol. 2017;53(3):413–27.

    Article  CAS  PubMed  Google Scholar 

  67. Maverakis E, Marzano AV, Le ST, Callen JP, Brüggen MC, Guenova E, et al. Pyoderma gangrenosum. Nat Rev Dis Primers. 2020;6(1):81.

    Article  PubMed  Google Scholar 

  68. Marzano AV, Fanoni D, Antiga E, Quaglino P, Caproni M, Crosti C, et al. Expression of cytokines, chemokines and other effector molecules in two prototypic autoinflammatory skin diseases, pyoderma gangrenosum and Sweet’s syndrome. Clin Exp Immunol. 2014;178(1):48–56.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Braswell SF, Kostopoulos TC, Ortega-Loayza AG. Pathophysiology of pyoderma gangrenosum (PG): an updated review. J Am Acad Dermatol. 2015;73(4):691–8.

    Article  CAS  PubMed  Google Scholar 

  70. Prajapati V, Man J, Brassard A. Pyoderma gangrenosum: common pitfalls in management and a stepwise, evidence-based, therapeutic approach. J Cutan Med Surg. 2009;13(Suppl 1):S2-11.

    Article  PubMed  Google Scholar 

  71. Hughes AP, Jackson JM, Callen JP. Clinical features and treatment of peristomal pyoderma gangrenosum. JAMA. 2000;284(12):1546–8.

    Article  CAS  PubMed  Google Scholar 

  72. Jolles S, Niclasse S, Benson E. Combination oral and topical tacrolimus in therapy-resistant pyoderma gangrenosum. Br J Dermatol. 1999;140(3):564–5.

    Article  CAS  PubMed  Google Scholar 

  73. Marzano AV, Trevisan V, Lazzari R, Crosti C. Pyoderma gangrenosum: study of 21 patients and proposal of a “clinicotherapeutic” classification. J Dermatol Treat. 2011;22(5):254–60.

    Article  Google Scholar 

  74. Brooklyn TN, Dunnill MG, Shetty A, Bowden JJ, Williams JD, Griffiths CE, et al. Infliximab for the treatment of pyoderma gangrenosum: a randomised, double blind, placebo controlled trial. Gut. 2006;55(4):505–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  75. Hubbard VG, Friedmann AC, Goldsmith P. Systemic pyoderma gangrenosum responding to infliximab and adalimumab. Br J Dermatol. 2005;152(5):1059–61.

    Article  CAS  PubMed  Google Scholar 

  76. Alkhouri N, Hupertz V, Mahajan L. Adalimumab treatment for peristomal pyoderma gangrenosum associated with Crohn’s disease. Inflamm Bowel Dis. 2009;15(6):803–6.

    Article  PubMed  Google Scholar 

  77. Vidal D, Puig L, Gilaberte M, Alomar A. Review of 26 cases of classical pyoderma gangrenosum: clinical and therapeutic features. J Dermatol Treat. 2004;15(3):146–52.

    Article  CAS  Google Scholar 

  78. Nousari HC, Lynch W, Anhalt GJ, Petri M. The effectiveness of mycophenolate mofetil in refractory pyoderma gangrenosum. Arch Dermatol. 1998;134(12):1509–11.

    Article  CAS  PubMed  Google Scholar 

  79. von den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol. 1997;137(6):1000–5.

    Article  PubMed  Google Scholar 

  80. Sardar P, Guha P, Das NK, Gharami RC, Majumdar S, Banerjee D, et al. Ulcerative pyoderma gangrenosum in mixed connective tissue disorder: a rare association and role of azathioprine in the management. Indian J Dermatol. 2011;56(5):600–2.

    Article  PubMed  PubMed Central  Google Scholar 

  81. Din RS, Tsiaras WG, Li DG, Mostaghimi A. Efficacy of systemic dapsone treatment for pyoderma gangrenosum: a retrospective review. J Drugs Dermatol. 2018;17(10):1058–60.

    CAS  PubMed  Google Scholar 

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

    Article  PubMed  PubMed Central  Google Scholar 

  83. Cohen PR, Kurzrock R. Sweet’s syndrome revisited: a review of disease concepts. Int J Dermatol. 2003;42(10):761–78.

    Article  PubMed  Google Scholar 

  84. von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol. 1994;31(4):535–56 (quiz 57–60).

    Article  PubMed  Google Scholar 

  85. Ali M, Duerksen DR. Ulcerative colitis and Sweet’s syndrome: a case report and review of the literature. Can J Gastroenterol. 2008;22(3):296–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  86. Cohen PR, Kurzrock R. Sweet’s syndrome: a review of current treatment options. Am J Clin Dermatol. 2002;3(2):117–31.

    Article  PubMed  Google Scholar 

  87. Suehisa S, Tagami H. Treatment of acute febrile neutrophilic dermatosis (Sweet’s syndrome) with colchicine. Br J Dermatol. 1981;105(4):483.

    Article  CAS  PubMed  Google Scholar 

  88. Amouri M, Masmoudi A, Ammar M, Boudaya S, Khabir A, Boudawara T, et al. Sweet’s syndrome: a retrospective study of 90 cases from a tertiary care center. Int J Dermatol. 2016;55(9):1033–9.

    Article  PubMed  Google Scholar 

  89. el Sherif AI, Bharija SC, Belhaj MS, Singh G. Dapsone in Sweet syndrome. Int J Dermatol. 1990;29(10):737.

    PubMed  Google Scholar 

  90. Bourke JF, Berth-Jones J, Graham-Brown RA. Sweet’s syndrome responding to cyclosporin. Br J Dermatol. 1992;127(1):36–8.

    Article  CAS  PubMed  Google Scholar 

  91. Seminario-Vidal L, Guerrero C, Sami N. Refractory Sweet’s syndrome successfully treated with rituximab. JAAD Case Rep. 2015;1(3):123–5.

    Article  PubMed  PubMed Central  Google Scholar 

  92. Agarwal A, Barrow W, Selim MA, Nicholas MW. Refractory subcutaneous Sweet syndrome treated with adalimumab. JAMA Dermatol. 2016;152(7):842–4.

    Article  PubMed  Google Scholar 

  93. Schmidt E, Kasperkiewicz M, Joly P. Pemphigus. Lancet. 2019;394(10201):882–94.

    Article  CAS  PubMed  Google Scholar 

  94. Svecova D. Pemphigus vulgaris: a clinical study of 44 cases over a 20-year period. Int J Dermatol. 2015;54(10):1138–44.

    Article  PubMed  Google Scholar 

  95. Chmurova N, Svecova D. Pemphigus vulgaris: a 11-year review. Bratisl Lek Listy. 2009;110(8):500–3.

    CAS  PubMed  Google Scholar 

  96. Kridin K, Zelber-Sagi S, Comaneshter D, Batat E, Cohen AD. Pemphigus and hematologic malignancies: a population-based study of 11,859 patients. J Am Acad Dermatol. 2018;78(6):1084-9.e1.

    Article  PubMed  Google Scholar 

  97. Kridin K. Pemphigus group: overview, epidemiology, mortality, and comorbidities. Immunol Res. 2018;66(2):255–70.

    Article  PubMed  Google Scholar 

  98. Heelan K, Mahar AL, Walsh S, Shear NH. Pemphigus and associated comorbidities: a cross-sectional study. Clin Exp Dermatol. 2015;40(6):593–9.

    Article  CAS  PubMed  Google Scholar 

  99. Seo JW, Park J, Lee J, Kim MY, Choi HJ, Jeong HJ, et al. A case of pemphigus vulgaris associated with ulcerative colitis. Intest Res. 2018;16(1):147–50.

    Article  PubMed  PubMed Central  Google Scholar 

  100. Kacar S, Sezgin O, Sahin T. Pemphigus vulgaris and ulcerative colitis. Am J Gastroenterol. 2002;97(2):507–8.

    Article  PubMed  Google Scholar 

  101. Prendiville JS, Israel DM, Wood WS, Dimmick JE. Oral pemphigus vulgaris associated with inflammatory bowel disease and herpetic gingivostomatitis in an 11-year-old girl. Pediatr Dermatol. 1994;11(2):145–50.

    Article  CAS  PubMed  Google Scholar 

  102. Kridin K, Zelber-Sagi S, Comaneshter D, Cohen AD. Ulcerative colitis associated with pemphigus: a population-based large-scale study. Scand J Gastroenterol. 2017;52(12):1360–4.

    Article  PubMed  Google Scholar 

  103. Takizawa H, Watanabe S, Takahashi M, Asakura H. Pemphigus vulgaris with asymptomatic pseudomembranous colitis. Am J Gastroenterol. 1996;91(8):1654–5.

    CAS  PubMed  Google Scholar 

  104. Leshem YA, Hodak E, David M, Anhalt GJ, Mimouni D. Successful treatment of pemphigus with biweekly 1-g infusions of rituximab: a retrospective study of 47 patients. J Am Acad Dermatol. 2013;68(3):404–11.

    Article  CAS  PubMed  Google Scholar 

  105. Anandan V, Jameela WA, Sowmiya R, Kumar MMS, Lavanya P. Rituximab: a magic bullet for pemphigus. J Clin Diagn Res. 2017;11(4):WC01–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  106. Lunardon L, Tsai KJ, Propert KJ, Fett N, Stanley JR, Werth VP, et al. Adjuvant rituximab therapy of pemphigus: a single-center experience with 31 patients. Arch Dermatol. 2012;148(9):1031–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  107. Kasperkiewicz M, Shimanovich I, Meier M, Schumacher N, Westermann L, Kramer J, et al. Treatment of severe pemphigus with a combination of immunoadsorption, rituximab, pulsed dexamethasone and azathioprine/mycophenolate mofetil: a pilot study of 23 patients. Br J Dermatol. 2012;166(1):154–60.

    Article  CAS  PubMed  Google Scholar 

  108. Antonelli E, Bassotti G, Tramontana M, Hansel K, Stingeni L, Ardizzone S, et al. Dermatological manifestations in inflammatory bowel diseases. J Clin Med. 2021;10(2):364.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  109. Thrash B, Patel M, Shah KR, Boland CR, Menter A. Cutaneous manifestations of gastrointestinal disease: part II. J Am Acad Dermatol. 2013;68(2):211.e1-233.e1 (quiz 44–6).

    Article  Google Scholar 

  110. Hagen JW, Swoger JM, Grandinetti LM. Cutaneous manifestations of Crohn disease. Dermatol Clin. 2015;33(3):417–31.

    Article  CAS  PubMed  Google Scholar 

  111. Roth N, Biedermann L, Fournier N, Butter M, Vavricka SR, Navarini AA, et al. Occurrence of skin manifestations in patients of the Swiss inflammatory bowel disease cohort study. PLoS ONE. 2019;14(1):e0210436.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  112. Cui RZ, Bruce AJ, Rogers RS. Recurrent aphthous stomatitis. Clin Dermatol. 2016;34(4):475–81.

    Article  PubMed  Google Scholar 

  113. Scully C, Porter S. Oral mucosal disease: recurrent aphthous stomatitis. Br J Oral Maxillofac Surg. 2008;46(3):198–206.

    Article  PubMed  Google Scholar 

  114. Vavricka SR, Gubler M, Gantenbein C, Spoerri M, Froehlich F, Seibold F, et al. Anti-TNF treatment for extraintestinal manifestations of inflammatory bowel disease in the Swiss IBD cohort study. Inflamm Bowel Dis. 2017;23(7):1174–81.

    Article  PubMed  Google Scholar 

  115. Greenstein AJ, Janowitz HD, Sachar DB. The extra-intestinal complications of Crohn’s disease and ulcerative colitis: a study of 700 patients. Medicine (Baltimore). 1976;55(5):401–12.

    Article  CAS  Google Scholar 

  116. Greuter T, Vavricka SR. Extraintestinal manifestations in inflammatory bowel disease—epidemiology, genetics, and pathogenesis. Expert Rev Gastroenterol Hepatol. 2019;13(4):307–17.

    Article  CAS  PubMed  Google Scholar 

  117. Karmiris K, Avgerinos A, Tavernaraki A, Zeglinas C, Karatzas P, Koukouratos T, et al. Prevalence and characteristics of extra-intestinal manifestations in a large cohort of Greek patients with inflammatory bowel disease. J Crohns Colitis. 2016;10(4):429–36.

    Article  PubMed  Google Scholar 

  118. Trost LB, McDonnell JK. Important cutaneous manifestations of inflammatory bowel disease. Postgrad Med J. 2005;81(959):580–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  119. Timani S, Mutasim DF. Skin manifestations of inflammatory bowel disease. Clin Dermatol. 2008;26(3):265–73.

    Article  PubMed  Google Scholar 

  120. Apgar JT. Newer aspects of inflammatory bowel disease and its cutaneous manifestations: a selective review. Semin Dermatol. 1991;10(3):138–47.

    CAS  PubMed  Google Scholar 

  121. Requena L, Requena C. Erythema nodosum. Dermatol Online J. 2002;8(1):4.

    PubMed  Google Scholar 

  122. Blake T, Manahan M, Rodins K. Erythema nodosum—a review of an uncommon panniculitis. Dermatol Online J. 2014;20(4):22376.

    PubMed  Google Scholar 

  123. Ubogy Z, Persellin RH. Suppression of erythema nodosum by indomethacin. Acta Derm Venereol. 1982;62(3):265–6.

    CAS  PubMed  Google Scholar 

  124. Lehman CW. Control of chronic erythema nodosum with naproxen. Cutis. 1980;26(1):66–7.

    CAS  PubMed  Google Scholar 

  125. Horio T, Danno K, Okamoto H, Miyachi Y, Imamura S. Potassium iodide in erythema nodosum and other erythematous dermatoses. J Am Acad Dermatol. 1983;9(1):77–81.

    Article  CAS  PubMed  Google Scholar 

  126. Elizaga FV. Erythema nodosum and indomethacin. Ann Intern Med. 1982;96(3):383.

    Article  CAS  PubMed  Google Scholar 

  127. Alloway JA, Franks LK. Hydroxychloroquine in the treatment of chronic erythema nodosum. Br J Dermatol. 1995;132(4):661–2.

    Article  CAS  PubMed  Google Scholar 

  128. Gilchrist H, Patterson JW. Erythema nodosum and erythema induratum (nodular vasculitis): diagnosis and management. Dermatol Ther. 2010;23(4):320–7.

    Article  PubMed  Google Scholar 

  129. Reddy H, Shipman AR, Wojnarowska F. Epidermolysis bullosa acquisita and inflammatory bowel disease: a review of the literature. Clin Exp Dermatol. 2013;38(3):225–9 (quiz 9–30).

    Article  CAS  PubMed  Google Scholar 

  130. Koga H, Prost-Squarcioni C, Iwata H, Jonkman MF, Ludwig RJ, Bieber K. Epidermolysis bullosa acquisita: the 2019 update. Front Med (Lausanne). 2018;5:362.

    Article  PubMed  Google Scholar 

  131. Chen M, Kim GH, Prakash L, Woodley DT. Epidermolysis bullosa acquisita: autoimmunity to anchoring fibril collagen. Autoimmunity. 2012;45(1):91–101.

    Article  CAS  PubMed  Google Scholar 

  132. Gupta R, Woodley DT, Chen M. Epidermolysis bullosa acquisita. Clin Dermatol. 2012;30(1):60–9.

    Article  PubMed  PubMed Central  Google Scholar 

  133. Chen M, O’Toole EA, Sanghavi J, Mahmud N, Kelleher D, Weir D, et al. The epidermolysis bullosa acquisita antigen (type VII collagen) is present in human colon and patients with Crohn’s disease have autoantibodies to type VII collagen. J Invest Dermatol. 2002;118(6):1059–64.

    Article  CAS  PubMed  Google Scholar 

  134. Ungureanu L, Cosgarea R, Alexandru Badea M, Florentina Vasilovici A, Cosgarea I, Corina ȘS. Cutaneous manifestations in inflammatory bowel disease (review). Exp Ther Med. 2020;20(1):31–7.

    PubMed  Google Scholar 

  135. Vodegel RM, Jonkman MF, Pas HH, de Jong MC. U-serrated immunodeposition pattern differentiates type VII collagen targeting bullous diseases from other subepidermal bullous autoimmune diseases. Br J Dermatol. 2004;151(1):112–8.

    Article  CAS  PubMed  Google Scholar 

  136. Kim JH, Kim YH, Kim SC. Epidermolysis bullosa acquisita: a retrospective clinical analysis of 30 cases. Acta Derm Venereol. 2011;91(3):307–12.

    Article  CAS  PubMed  Google Scholar 

  137. Iranzo P, Herrero-González JE, Mascaró-Galy JM, Suárez-Fernández R, España A. Epidermolysis bullosa acquisita: a retrospective analysis of 12 patients evaluated in four tertiary hospitals in Spain. Br J Dermatol. 2014;171(5):1022–30.

    Article  CAS  PubMed  Google Scholar 

  138. Gürcan HM, Ahmed AR. Current concepts in the treatment of epidermolysis bullosa acquisita. Expert Opin Pharmacother. 2011;12(8):1259–68.

    Article  PubMed  CAS  Google Scholar 

  139. Iwata H, Vorobyev A, Koga H, Recke A, Zillikens D, Prost-Squarcioni C, et al. Meta-analysis of the clinical and immunopathological characteristics and treatment outcomes in epidermolysis bullosa acquisita patients. Orphanet J Rare Dis. 2018;13(1):153.

    Article  PubMed  PubMed Central  Google Scholar 

  140. Cleynen I, Van Moerkercke W, Billiet T, Vandecandelaere P, Vande Casteele N, Breynaert C, et al. Characteristics of skin lesions associated with anti-tumor necrosis factor therapy in patients with inflammatory bowel disease: a cohort study. Ann Intern Med. 2016;164(1):10–22.

    Article  PubMed  Google Scholar 

  141. Fréling E, Baumann C, Cuny JF, Bigard MA, Schmutz JL, Barbaud A, et al. Cumulative incidence of, risk factors for, and outcome of dermatological complications of anti-TNF therapy in inflammatory bowel disease: a 14-year experience. Am J Gastroenterol. 2015;110(8):1186–96.

    Article  PubMed  CAS  Google Scholar 

  142. Rahier JF, Buche S, Peyrin-Biroulet L, Bouhnik Y, Duclos B, Louis E, et al. Severe skin lesions cause patients with inflammatory bowel disease to discontinue anti-tumor necrosis factor therapy. Clin Gastroenterol Hepatol. 2010;8(12):1048–55.

    Article  PubMed  Google Scholar 

  143. Fiorino G, Danese S, Pariente B, Allez M. Paradoxical immune-mediated inflammation in inflammatory bowel disease patients receiving anti-TNF-α agents. Autoimmun Rev. 2014;13(1):15–9.

    Article  CAS  PubMed  Google Scholar 

  144. Hu JZ, Billings SD, Yan D, Fernandez AP. Histologic comparison of tumor necrosis factor-α inhibitor-induced psoriasis and psoriasis vulgaris. J Am Acad Dermatol. 2020;83(1):71–7.

    Article  CAS  PubMed  Google Scholar 

  145. Brown G, Wang E, Leon A, Huynh M, Wehner M, Matro R, et al. Tumor necrosis factor-α inhibitor-induced psoriasis: systematic review of clinical features, histopathological findings, and management experience. J Am Acad Dermatol. 2017;76(2):334–41.

    Article  CAS  PubMed  Google Scholar 

  146. Collamer AN, Guerrero KT, Henning JS, Battafarano DF. Psoriatic skin lesions induced by tumor necrosis factor antagonist therapy: a literature review and potential mechanisms of action. Arthritis Rheum. 2008;59(7):996–1001.

    Article  CAS  PubMed  Google Scholar 

  147. Collamer AN, Battafarano DF. Psoriatic skin lesions induced by tumor necrosis factor antagonist therapy: clinical features and possible immunopathogenesis. Semin Arthritis Rheum. 2010;40(3):233–40.

    Article  CAS  PubMed  Google Scholar 

  148. Li SJ, Perez-Chada LM, Merola JF. TNF inhibitor-induced psoriasis: proposed algorithm for treatment and management. J Psoriasis Psoriatic Arthritis. 2019;4(2):70–80.

    Article  PubMed  Google Scholar 

  149. Cullen G, Kroshinsky D, Cheifetz AS, Korzenik JR. Psoriasis associated with anti-tumour necrosis factor therapy in inflammatory bowel disease: a new series and a review of 120 cases from the literature. Aliment Pharmacol Ther. 2011;34(11–12):1318–27.

    Article  CAS  PubMed  Google Scholar 

  150. Andrisani G, Marzo M, Celleno L, Guidi L, Papa A, Gasbarrini A, et al. Development of psoriasis scalp with alopecia during treatment of Crohn’s disease with infliximab and rapid response to both diseases to ustekinumab. Eur Rev Med Pharmacol Sci. 2013;17(20):2831–6.

    CAS  PubMed  Google Scholar 

  151. Ezzedine K, Visseaux L, Cadiot G, Brixi H, Bernard P, Reguiai Z. Ustekinumab for skin reactions associated with anti-tumor necrosis factor-α agents in patients with inflammatory bowel diseases: a single-center retrospective study. J Dermatol. 2019;46(4):322–7.

    Article  CAS  PubMed  Google Scholar 

  152. Tillack C, Ehmann LM, Friedrich M, Laubender RP, Papay P, Vogelsang H, et al. Anti-TNF antibody-induced psoriasiform skin lesions in patients with inflammatory bowel disease are characterised by interferon-γ-expressing Th1 cells and IL-17A/IL-22-expressing Th17 cells and respond to anti-IL-12/IL-23 antibody treatment. Gut. 2014;63(4):567–77.

    Article  CAS  PubMed  Google Scholar 

  153. Gregoriou S, Kazakos C, Christofidou E, Kontochristopoulos G, Vakis G, Rigopoulos D. Pustular psoriasis development after initial ustekinumab administration in chronic plaque psoriasis. Eur J Dermatol. 2011;21(1):104–5.

    Article  PubMed  Google Scholar 

  154. Adegbola SO, Pisani A, Sahnan K, Tozer P, Ellul P, Warusavitarne J. Medical and surgical management of perianal Crohn’s disease. Ann Gastroenterol. 2018;31(2):129–39.

    PubMed  PubMed Central  Google Scholar 

  155. Stringer EE, Nicholson TJ, Armstrong D. Efficacy of topical metronidazole (10 percent) in the treatment of anorectal Crohn’s disease. Dis Colon Rectum. 2005;48(5):970–4.

    Article  PubMed  Google Scholar 

  156. Hart AL, Plamondon S, Kamm MA. Topical tacrolimus in the treatment of perianal Crohn’s disease: exploratory randomized controlled trial. Inflamm Bowel Dis. 2007;13(3):245–53.

    Article  PubMed  Google Scholar 

  157. Hughes LE. Surgical pathology and management of anorectal Crohn’s disease. J R Soc Med. 1978;71(9):644–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  158. Bouguen G, Trouilloud I, Siproudhis L, Oussalah A, Bigard MA, Bretagne JF, et al. Long-term outcome of non-fistulizing (ulcers, stricture) perianal Crohn’s disease in patients treated with infliximab. Aliment Pharmacol Ther. 2009;30(7):749–56.

    Article  CAS  PubMed  Google Scholar 

  159. Vogel JD, Johnson EK, Morris AM, Paquette IM, Saclarides TJ, Feingold DL, et al. Clinical practice guideline for the management of anorectal abscess, fistula-in-ano, and rectovaginal fistula. Dis Colon Rectum. 2016;59(12):1117–33.

    Article  PubMed  Google Scholar 

  160. Thia KT, Mahadevan U, Feagan BG, Wong C, Cockeram A, Bitton A, et al. Ciprofloxacin or metronidazole for the treatment of perianal fistulas in patients with Crohn’s disease: a randomized, double-blind, placebo-controlled pilot study. Inflamm Bowel Dis. 2009;15(1):17–24.

    Article  PubMed  Google Scholar 

Download references

Author information

Author notes

  1. Kyla Pagani and Danitza Lukac have contributed equally to this work.

Authors and Affiliations

  1. Department of Dermatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

    Kyla Pagani, Danitza Lukac & Jean S. McGee

  2. Nova Southeastern University Dr. Kiran C. Patel College of Osteopathic Medicine, Ft. Lauderdale, FL, USA

    Aashni Bhukhan

Authors
  1. Kyla Pagani
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Danitza Lukac
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Aashni Bhukhan
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jean S. McGee
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jean S. McGee.

Ethics declarations

Funding

There is no funding for this manuscript.

Conflict of interest

The authors have no conflicts of interest to declare.

Ethics approval

Not applicable.

Consent to participate/publish

Not applicable.

Code availability

Not applicable.

Author contributions

All authors contributed to the manuscript conception and design. Material preparation, data collection and analysis were performed by all authors. The first draft of the manuscript was written by KP, DL, and AB and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Data availability

Our manuscript has no associated data.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Pagani, K., Lukac, D., Bhukhan, A. et al. Cutaneous Manifestations of Inflammatory Bowel Disease: A Basic Overview. Am J Clin Dermatol 23, 481–497 (2022). https://doi.org/10.1007/s40257-022-00689-w

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40257-022-00689-w

Search

Navigation