Nonspecific oral mucositis in a patient with Crohn's disease

Abstract

Background A wide variety of oral lesions have been reported in Crohn's disease (CD), with a reported prevalence of 20% to 50%. We report a case of lip swelling and severe mucositis affecting the lips and the oral mucosa in a patient with CD. Case Summary A 48-year-old female presented with a 2-year history of painful gingiva and lips. She had seen 26 doctors for this condition and had undergone multiple biopsies with nonspecific results, including a most recent perioral biopsy showing spongiotic dermatitis with neutrophils and eosinophils and negative results on direct immunofluorescence (DIF). Attempted treatments included chlorhexidine rinse, dexamethasone rinse, azathioprine, an increase in the infliximab dose that she had been taking for CD, and prednisone. Among these treatments, only prednisone helped reduce the severity of the symptoms. Past medical history included CD, celiac disease, and meningioma. Medications included infliximab every 8 weeks for 7 years, azathioprine, amitriptyline, sertraline, and pantoprazole. Her CD had been well controlled, and multiple endoscopies and colonoscopies after the onset of the oral lesions had confirmed that there were no current CD lesions. Examination revealed swelling and generalized erythema with multiple ulcerations and blisters of the lips, generalized erythema on all facial gingiva and vestibules, and a slight hyperplastic appearance of the anterior facial gingiva simulating orofacial granulomatosis. Biopsies had been performed previously on perioral cutaneous tissues. We performed biopsy of oral mucosa, and the result, again, indicated nonspecific inflammation with a negative result on DIF. The oral lesions subsequently responded relatively well to systemic steroids. Conclusions This case demonstrates the simultaneous presentation of multiple conditions. Oral biopsy revealed a solitary focus of granulomatous inflammation, indicative of orofacial granulomatosis. However, the biopsied lesion was also unusual in that inflammation extended into the epithelium, a relatively unusual feature for a granulomatous inflammatory disease and a pattern more commonly seen in vesiculobullous eruptions. Because this patient's CD was well controlled, it is difficult to believe that these oral lesions were associated with CD activity. One reasonable assumption is that the long-time treatment of the patient's CD with infliximab might have hindered histologic and immunologic identification to establish a more definitive diagnosis.