Pediatric Patients With Orofacial Granulomatosis Likely To Develop Intestinal Crohn Disease

Orofacial granulomatosis (OFG) is a rare mucocutaneous disorder characterized by persistent granulomatous perioral inflammation in the absence of systemic disease. There is continued debate regarding whether OFG is a distinct clinical disorder or a manifestation of orofacial Crohn disease (CD) given indistinguishable clinical and histologic features. We aimed to determine if there is a relationship between a diagnosis of biopsy-proven OFG in the absence of systemic disease and the subsequent diagnosis of intestinal CD in a pediatric patient population. A retrospective chart review from 2000 to 2018 at a single tertiary pediatric hospital was performed. Seven patients met inclusion criteria of receiving a diagnosis of OFG in the absence of previous or concurrent diagnosis of inflammatory bowel disease. Four patients were subsequently diagnosed with intestinal CD during the study period with a median time to diagnosis of 36 months (range 4-72 months). The remaining three patients have no diagnosis of intestinal CD to date with a median follow-up time of 3 months (range 2-17 months). Similar to previous studies, our case series demonstrates that pediatric patients diagnosed with OFG are likely to receive a subsequent diagnosis of intestinal CD. At minimum, Gastroenterology evaluation is warranted with long-term monitoring for any signs of intestinal CD.