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Dermatologic and ocular manifestations of inflammatory bowel disease

Mark A Peppercorn, MD
Adam S Cheifetz, MD
Section Editors
Paul Rutgeerts, MD, PhD, FRCP
Jonathan Trobe, MD
Deputy Editor
Shilpa Grover, MD, MPH


There are several extrahepatic manifestations of inflammatory bowel disease (IBD). These manifestations vary in severity and can be more debilitating than the underlying IBD. While some extraintestinal manifestations parallel the disease activity of IBD (eg, erythema nodosum, episcleritis, and Sweet syndrome), the course of others (eg, pyoderma gangrenosum, uveitis) is independent of intestinal inflammation.

This topic will review the skin and eye manifestations of IBD. The clinical manifestations, diagnosis, and management of ulcerative colitis and Crohn disease are discussed separately. (See "Clinical manifestations, diagnosis, and prognosis of ulcerative colitis in adults" and "Management of mild to moderate ulcerative colitis" and "Management of severe ulcerative colitis" and "Clinical manifestations, diagnosis and prognosis of Crohn disease in adults" and "Overview of the medical management of mild to moderate Crohn disease in adults" and "Overview of the medical management of severe or refractory Crohn disease in adults".)


The prevalence of extraintestinal manifestations in patients with IBD has not been extensively studied. However, data suggest that 6 to 40 percent of patients with IBD have one or more extraintestinal manifestation [1,2]. Up to 15 percent of patients have a cutaneous manifestation of IBD [3,4]. Ocular manifestations of IBD occur in 4 to 10 percent of patients and may be more likely to occur in patients with Crohn disease as compared with ulcerative colitis [5,6].


The pathogenesis of extraintestinal manifestations in patients with IBD is incompletely understood. However, it is hypothesized that the diseased gastrointestinal mucosa may trigger an immune response at the extraintestinal site due to shared epitopes [7]. Triggers of the autoimmune response in certain organs may be influenced by genetic factors. Associations of extraintestinal manifestations of IBD with major histocompatibility complex loci have also been demonstrated [8]. As an example, HLA-B27 and HLA-B58 are associated with ocular inflammation in patients with IBD.


Erythema nodosum — Erythema nodosum is the most common dermatologic manifestation of inflammatory bowel disease (IBD), occurring in 3 to 10 percent of patients with ulcerative colitis and 4 to 15 percent of patients with Crohn disease [9]. Lesions typically consist of raised, tender, red or violet subcutaneous nodules that are 1 to 5 cm in diameter (picture 1). The nodules are most commonly located on the extensor surfaces of the extremities, particularly over the anterior tibial area. Biopsy of these lesions shows focal panniculitis. However, the diagnosis is most often clinical, and biopsy is required only in atypical cases (eg, patients with no lesions on the legs, persistence beyond six to eight weeks, or the development of ulceration). (See "Erythema nodosum", section on 'Clinical features'.)


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Literature review current through: Oct 2015. | This topic last updated: Sep 22, 2015.
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  1. Bernstein CN, Blanchard JF, Rawsthorne P, Yu N. The prevalence of extraintestinal diseases in inflammatory bowel disease: a population-based study. Am J Gastroenterol 2001; 96:1116.
  2. Ricart E, Panaccione R, Loftus EV Jr, et al. Autoimmune disorders and extraintestinal manifestations in first-degree familial and sporadic inflammatory bowel disease: a case-control study. Inflamm Bowel Dis 2004; 10:207.
  3. Vavricka SR, Brun L, Ballabeni P, et al. Frequency and risk factors for extraintestinal manifestations in the Swiss inflammatory bowel disease cohort. Am J Gastroenterol 2011; 106:110.
  4. 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; 55:401.
  5. Mintz R, Feller ER, Bahr RL, Shah SA. Ocular manifestations of inflammatory bowel disease. Inflamm Bowel Dis 2004; 10:135.
  6. Salmon JF, Wright JP, Murray AD. Ocular inflammation in Crohn's disease. Ophthalmology 1991; 98:480.
  7. 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; 98:464.
  8. Orchard TR, Chua CN, Ahmad T, et al. Uveitis and erythema nodosum in inflammatory bowel disease: clinical features and the role of HLA genes. Gastroenterology 2002; 123:714.
  9. Farhi D, Cosnes J, Zizi N, et al. Significance of erythema nodosum and pyoderma gangrenosum in inflammatory bowel diseases: a cohort study of 2402 patients. Medicine (Baltimore) 2008; 87:281.
  10. Powell FC, Schroeter AL, Su WP, Perry HO. Pyoderma gangrenosum: a review of 86 patients. Q J Med 1985; 55:173.
  11. Keltz M, Lebwohl M, Bishop S. Peristomal pyoderma gangrenosum. J Am Acad Dermatol 1992; 27:360.
  12. Thornton JR, Teague RH, Low-Beer TS, Read AE. Pyoderma gangrenosum and ulcerative colitis. Gut 1980; 21:247.
  13. Agarwal A, Andrews JM. Systematic review: IBD-associated pyoderma gangrenosum in the biologic era, the response to therapy. Aliment Pharmacol Ther 2013; 38:563.
  14. Travis S, Innes N, Davies MG, et al. Sweet's syndrome: an unusual cutaneous feature of Crohn's disease or ulcerative colitis. The South West Gastroenterology Group. Eur J Gastroenterol Hepatol 1997; 9:715.
  15. Uihlein LC, Brandling-Bennett HA, Lio PA, Liang MG. Sweet syndrome in children. Pediatr Dermatol 2012; 29:38.
  16. André M, Aumaître O. [Aseptic abscesses syndrome]. Rev Med Interne 2011; 32:678.
  17. Ito T, Sato N, Yamazaki H, et al. A case of aseptic abscesses syndrome treated with corticosteroids and TNF-alpha blockade. Mod Rheumatol 2013; 23:195.
  18. Truchuelo MT, Alcántara J, Vano-Galván S, et al. Bowel-associated dermatosis-arthritis syndrome: another cutaneous manifestation of inflammatory intestinal disease. Int J Dermatol 2013; 52:1596.
  19. Chiu G, Rajapakse CN. Cutaneous polyarteritis nodosa and ulcerative colitis. J Rheumatol 1991; 18:769.
  20. Akbulut S, Ozaslan E, Topal F, et al. Ulcerative colitis presenting as leukocytoclastic vasculitis of skin. World J Gastroenterol 2008; 14:2448.
  21. Burgdorf W. Cutaneous manifestations of Crohn's disease. J Am Acad Dermatol 1981; 5:689.
  22. Tweedie JH, McCann BG. Metastatic Crohn's disease of thigh and forearm. Gut 1984; 25:213.
  23. Abide JM. Metastatic Crohn disease: clearance with metronidazole. J Am Acad Dermatol 2011; 64:448.
  24. Konrad A, Seibold F. Response of cutaneous Crohn's disease to infliximab and methotrexate. Dig Liver Dis 2003; 35:351.
  25. Reddy H, Shipman AR, Wojnarowska F. Epidermolysis bullosa acquisita and inflammatory bowel disease: a review of the literature. Clin Exp Dermatol 2013; 38:225.
  26. Raab B, Fretzin DF, Bronson DM, et al. Epidermolysis bullosa acquisita and inflammatory bowel disease. JAMA 1983; 250:1746.
  27. Najarian DJ, Gottlieb AB. Connections between psoriasis and Crohn's disease. J Am Acad Dermatol 2003; 48:805.
  28. Lee FI, Bellary SV, Francis C. Increased occurrence of psoriasis in patients with Crohn's disease and their relatives. Am J Gastroenterol 1990; 85:962.
  29. Hughes S, Williams SE, Turnberg LA. Crohn's disease and psoriasis. N Engl J Med 1983; 308:101.
  30. Kappelman MD, Farkas DK, Long MD, et al. Risk of cancer in patients with inflammatory bowel diseases: a nationwide population-based cohort study with 30 years of follow-up evaluation. Clin Gastroenterol Hepatol 2014; 12:265.
  31. Singh S, Nagpal SJ, Murad MH, et al. Inflammatory bowel disease is associated with an increased risk of melanoma: a systematic review and meta-analysis. Clin Gastroenterol Hepatol 2014; 12:210.
  32. Long MD, Martin CF, Pipkin CA, et al. Risk of melanoma and nonmelanoma skin cancer among patients with inflammatory bowel disease. Gastroenterology 2012; 143:390.
  33. Abbas AM, Almukhtar RM, Loftus EV Jr, et al. Risk of melanoma and non-melanoma skin cancer in ulcerative colitis patients treated with thiopurines: a nationwide retrospective cohort. Am J Gastroenterol 2014; 109:1781.
  34. Ariyaratnam J, Subramanian V. Association between thiopurine use and nonmelanoma skin cancers in patients with inflammatory bowel disease: a meta-analysis. Am J Gastroenterol 2014; 109:163.
  35. Petrelli EA, McKinley M, Troncale FJ. Ocular manifestations of inflammatory bowel disease. Ann Ophthalmol 1982; 14:356.
  36. Lyons JL, Rosenbaum JT. Uveitis associated with inflammatory bowel disease compared with uveitis associated with spondyloarthropathy. Arch Ophthalmol 1997; 115:61.
  37. Knox DL, Snip RC, Stark WJ. The keratopathy of Crohn's disease. Am J Ophthalmol 1980; 90:862.
  38. Schulman MF, Sugar A. Peripheral corneal infiltrates in inflammatory bowel disease. Ann Ophthalmol 1981; 13:109.
  39. Ruby AJ, Jampol LM. Crohn's disease and retinal vascular disease. Am J Ophthalmol 1990; 110:349.
  40. Kaneko E, Nawano M, Honda N, et al. Ulcerative colitis complicated by idiopathic central serous chorioretinopathy with bullous retinal detachment. Dig Dis Sci 1985; 30:896.