Sunday, January 12, 2014

Extraintestinal Inflammatory Bowel Disease

Unusual Manifestations of IBD

Crohn’s disease tends to affect the lower intestine in non-contiguous bands of inflammation.   Similarly, Ulcerative Colitis tends to present as a contiguous band of inflammation within the large intestine, potentially including the rectum.  These are the “textbook” presentations of IBD, but there are numerous atypical manifestations of IBD as well.  There are frequent extra-intestinal involvements, such as perianal disease, ocular and joint issues, and co-morbid disease (like psoriasis and rheumatoid arthritis), but there are also manifestations that are rare enough they may not be readily associated with IBD.  This blog post looks at a few of them.

Genital IBD

While fistulae involving the urinary tract are not uncommon, non-fistulizing complications involving both male and female genitals can also occur.  In terms of the penis and scrotum, Crohn’s disease can cause metatstatic lesions that are not fistula related.  Similarly, vulvar Crohn’s disease can cause lesions on the vulva and swelling of the labia.  While exact figures of occurrence are not available, these manifestations are very rare and are frequently misdiagnosed as venereal disease.(1,2)

IBD and the Skin

While IBD treatments have been known to cause a skin rash from drug-induced lupus as well as psoriasis, IBD itself can manifest in the skin.  Both Ulcerative Colitis and Crohn’s can manifest as Pyoderma gangrenosum, necrotic ulcers that generally appear as small bug-bite sized inflammation and progress to large ulcerations if untreated.  Erythema nodosum presents itself as red, raised areas on the shins due to inflammation of the underlying fat cells and can be from either variant of IBD.(3)

Upper-GI IBD

IBD is generally associated with the lower portions of the gastrointestinal system, but can impact the upper GI as well.  Starting with the oral cavity, IBD can manifest as aphthous stomatitis (canker sores), which are recurrent, painful inflammation of the mucosal areas in the mouth – generally on the inner cheeks, back of the lips and gums.  Pyostomatitis vegetans results in oral abscess formation and can be associated with Ulcerative Colitis.(4)  In one study of children with Crohn’s, 42% were found to have oral manifestations as follows:

Oral CD was found in 20 patients (41.7%). Oral findings included mucogingivitis (12 patients), mucosal tags (4 patients), deep ulceration (4 patients), cobblestoning (3 patients), lip swelling (3 patients), and pyostomatitis vegetans (1 patient).(5)

At the next stage, esophageal disease is less frequent, impacting .2% of individuals with Crohn’s disease.  The esophagus can become laden with ulcers and erosions, and inflammation can be present the same as it is in the intestines.  Similarly, and more problematic, structuring of the esophagus can impact diet and general quality of life.(6)

Gastroduodenal IBD is another fairly uncommon variant (and generally contiguous with lower GI Crohn’s).  Impacting the stomach and the first part of the small intestine, this presentation can have the same issues as lower GI Crohn’s – structuring, ulcerations, and even fistula formation.(7)  While upper GI disease is believed to impact 3% of patients, they tend to have more abdominal pain and to have a younger age of onset.  With rare exceptions, lower GI impact will occur at some point in the disease progression.(8)

There are other, rare manifestations as well, ranging from nasal involvement to metastatic pulmonary IBD.  Because it can present in just about any location, IBD that is not found initially in the intestines may take longer to identify and properly diagnose.  Fortunately, the common systemic treatments (ranging from corticosteroids to anti-TNF-alpha drugs) are generally the recommended course of action for any extra-intestinal manifestations.

Bottom Line

·         Crohn’s and Ulcerative Colitis can manifest in the skin, upper GI tract, genitals, and just about anywhere on the body.
·         Extraintestinal complications make diagnosis difficult, but the treatment for extraintestinal IBD is largely the same as for lower GI IBD.

1.       Slaney, G., S. Muller, J. Clay, A. H. Sumathipala, P. Hillenbrand, and H. Thompson. "Crohn's disease involving the penis." Gut 27, no. 3 (1986): 329-333.
2.       Vettraino, Ivana M., and Diane F. Merritt. "Crohn's disease of the vulva." The American journal of dermatopathology 17, no. 4 (1995): 410-413.
3.       Trost, L. B., and J. K. McDonnell. "Important cutaneous manifestations of inflammatory bowel disease." Postgraduate medical journal 81, no. 959 (2005): 580-585.
4.       Storwick, Gregory S., Marie B. Prihoda, Ronald J. Fulton, and William S. Wood. "Pyodermatitis-pyostomatitis vegetans: a specific marker for inflammatory bowel disease." Journal of the American Academy of Dermatology31, no. 2 (1994): 336-341.
5.       Harty, Sinead, Padraig Fleming, Marion Rowland, Ellen Crushell, Michael McDermott, Brendan Drumm, and Billy Bourke. "A prospective study of the oral manifestations of Crohn’s disease." Clinical Gastroenterology and Hepatology 3, no. 9 (2005): 886-891.
6.       Decker, G. Anton G., Edward V. Loftus, Tousif M. Pasha, William J. Tremaine, and William J. Sandborn. "Crohn's disease of the esophagus: clinical features and outcomes." Inflammatory bowel diseases 7, no. 2 (2001): 113-119.
7.       Yamamoto, T., R. N. Allan, and M. R. B. Keighley. "An audit of gastroduodenal Crohn disease: clinicopathologic features and management." Scandinavian journal of gastroenterology 34, no. 10 (1999): 1019-1024.

8.       Rutgeerts, Paul, E. Onette, Gaston Vantrappen, Karel Geboes, L. Broeckaert, and L. Talloen. "Crohn's disease of the stomach and duodenum: a clinical study with emphasis on the value of endoscopy and endoscopic biopsies."Endoscopy 12, no. 06 (1980): 288-294.

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