Severe refractory orofacial Crohn's disease: report of a case

Oral involvement is common in patients with Crohn's disease (CD) and can precede intestinal symptoms, making diagnosis difficult. We report a case of severe orofacial CD. A 41-year-old woman presented with palate and tongue ulcers. Biopsies showed acute inflammation with ulcer. Colonoscopy demonstrated ascending colon ulceration. Biopsies revealed acute colitis and mild architectural distortion. Prednisone was started but the symptoms recurred with taper; steroids were resumed and infliximab (IFX) 5 mg/kg was infused. After improvement, oral pain and weight loss returned. A G tube was placed. Mercaptopurine was started at 1.5 mg/kg per day. IFX was increased to 10 mg/kg. Debridement of the oral ulcers and a skin graft to the lips was performed. Pathology from oral and facial lesions was consistent with granulation tissue and fibrosis with chronic inflammation. She was readmitted several months later for weight loss and dehydration. Abdominal pain, distension, and feculent drainage developed around the G tube. Repeat computed tomography (CT) scan demonstrated pneumatosis. Laparotomy revealed purulent drainage from a perforated segment of sigmoid colon. Histology was consistent with perforated CD. Despite ventilatory and hemodynamic support and broad-spectrum antibiotics, the patient died 1 week later. Our case highlights the difficulty in diagnosing and managing orofacial CD. In this case, medical treatment was initiated based on a high index of suspicion. CD was only confirmed after intestinal resection very late in the disease course. Treatment of orofacial CD includes topical or systemic steroids, immunomodulators, and anti-tumor necrosis factor (TNF) therapies. As our case demonstrated, patients can be refractory to therapy.