Dermatologic and ocular manifestations of inflammatory bowel disease
- Mark A Peppercorn, MD
Mark A Peppercorn, MD
- Professor of Medicine
- Harvard Medical School
- Adam S Cheifetz, MD
Adam S Cheifetz, MD
- Associate Professor of Medicine
- Harvard Medical School
- Section Editors
- Paul Rutgeerts, MD, PhD, FRCP
Paul Rutgeerts, MD, PhD, FRCP
- Section Editor — Inflammatory Bowel Disease
- Emeritus Professor of Medicine
- University Hospital, Leuven, Belgium
- Jonathan Trobe, MD
Jonathan Trobe, MD
- Section Editor — Ophthalmology
- Professor of Ophthalmology and Visual Sciences
- Professor of Neurology
- University of Michigan Kellogg Eye Center
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 in adults" and "Management of severe ulcerative colitis in adults" 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 . 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 . 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 . 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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- DERMATOLOGIC DISEASE
- Erythema nodosum
- Pyoderma gangrenosum
- Rare dermatologic diseases
- - Neutrophilic dermatoses
- - Cutaneous vasculitis
- - Metastatic Crohn disease
- - Epidermolysis bullosa acquisita
- Associated conditions and complications
- - Psoriasis
- - Skin cancer
- OCULAR DISEASE
- Indications for urgent evaluation
- Rare ocular diseases
- - Keratopathy
- - Retinopathy
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS