Introduction
Inflammatory bowel disease (IBD)
comprises two major pathological conditions affecting the
gastrointestinal tract, i.e., Crohn´s disease (CD) and ulcerative
colitis (UC). CD can affect any part of the GI, while UC affects the
large intestine. IBD can also involve many other organs of the body from
mouth to anus, including the oral cavity.
The incidence of IBD is increasing, especially in newly industrialized
countries.1 In Europe, the incidence of CD ranges
between 0.4 and 22.8 per 100 000 people per year and UC between 2.4 and
44.0 per 100 000 people per year.2 The prevalence is
approximately 0.2% of the European population.2
Patients with IBD suffer from abdominal pain, diarrhea, weight loss,
secondary anemia, and fistulas.3 Oral manifestations
may appear years before systemic symptoms.3 These
include aphthous ulcers, mucogingivitis, lip swelling, angular
cheilitis, mucosal tags, cobblestoning, and deep linear ulcerations.
Histopathologically, granulomatous lesions can be seen. The etiology of
IBD remains unknown, but it is believed to be multifactorial, involving
genetic, immunologic, and environmental
factors.2,3,4,5 IBD should be considered a systemic
disease, since extraintestinal manifestations (EIMs) present in 5% to
50% of all IBD cases. EIMs can affect nearly every organ and might
appear prior to the first diagnosis of IBD, simultaneously, or after
resection of the affected bowel segment. EIMs, such as primary
sclerosing cholangitis (PSC) as a hepatobiliary manifestation, must be
recognized early to prevent severe morbidity and mortality. We report
the case of a young female with oral manifestation four years before
diagnosis of Crohn´s disease and subsequent PSC.