Introduction Orofacial Granulomatosis (OFG) is a rare chronic inflammatory condition of unknown aetiology, characterised by lip swelling, orofacial erythema and ulceration. A proportion of OFG patients present with perianal disease in conjunction with their oral disease (‘top and tail’ disease). Perianal disease occurs in approximately one-third of Crohn’s Disease (CD) patients and is associated with significant morbidity and a more severe disease course.1Perianal disease has been shown to occur in 12% of patients with ileal CD (L1), 41–92% of colonic CD (L2) and 15% of ileocolonic disease (L3).2
Method We retrospectively analysed a database of OFG patients. Patients with perianal disease were identified and compared to patients without perianal disease. The Montreal classification was used to classify the sites of patient’s CD. We set out to determine how many of our OFG patients had concurrent perianal disease and how many of them developed intestinal CD.
Results 263 patients with OFG were identified, of which 208 patients (79.09%) had OFG only and 55 patients (20.91%) had concurrent intestinal CD. 36 patients (13.69%) had intestinal CD and no perianal disease. 19 patients ((7.22%) 13 male, median age 38 (IQR 25–49)) had intestinal CD and concurrent perianal disease.
Within the perianal group, all patients had concurrent intestinal CD. The commonest sites were colonic (L2)(8/19; 42.11%) and ileocolonic (L3)(8/19; 42.11%). The ileum (L1) was affected in 1 patient (1/19; 5.26%) and 2 patients had concomitant upper gastrointestinal CD with ileocolonic disease (L3+L4)(2/19; 10.53%).
The presence of OFG and perianal disease significantly increased the chances of developing intestinal CD (OR = 222, p = 0.0002, 2-tail Fisher Test).
In the perianal group, 11/19 patients (11/19; 57.89%) were diagnosed with CD prior to developing OFG. The median time to diagnosis of OFG was 10 years after the diagnosis of intestinal CD.
Conclusion Perianal disease in Crohn’s disease is common and is associated with a more severe disease course.1Perianal disease in OFG patients is less common, however, where it does occur it is always associated with intestinal CD in our cohort. Therefore, these patients should be investigated accordingly.
The diagnosis of OFG was usually made later in the course of intestinal Crohn’s suggesting it is a later development.
Disclosure of interest None Declared.
Ardizzone S, Porro GB. Perianal Crohn’s disease: overview. Dig Liver Dis. 2007;39:957–958
Schwartz D, Loftus EV, Tremaine WJ, et al. The natural history of fistulising Crohn’s disease in Olmsted County, Minnesota. Gastroenterology 2002;122:875–880
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