Severe Refractory Orofacial Crohn’s Disease: Report of a Case

Abstract

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.

Experience with anti-TNF-α therapy for orofacial granulomatosis

“…This report describes the largest case series to date for patients with OFG+ ⁄ ) CD treated with anti-TNF-a therapy. Only a few cases and small case series (up to two patients) of the use of anti-TNF-a for OFG have previously been described, mostly reporting good shortterm benefit but with safety concerns raised (15)(16)(17)(18)(19)(20)(21)(22)(23). Seven per cent of patients referred to our clinic required anti-TNF-a therapy.…”

“…Anti‐TNF‐α therapy is a rational alternative for refractory OFG given the documented efficacy of infliximab (IFX) and adalimumab (ADA) for the treatment of CD, a condition characterized by elevated TNF‐alpha levels and sharing similar histopathological features with OFG (14). The use of IFX for patients with refractory OFG is relatively unexplored with only a small number of case reports published since an initial report of use in OFG in 2001 (15–23). There are two reported cases of the use of ADA in OFG (16, 20).…”

Cutaneous Manifestations in Patients With Inflammatory Bowel Diseases

“…For more severe or refractory orofacial CD, treatment should include systemic steroids, immunomodulators, and anti-TNF-a therapy. 42,43 In patients with colostomies or ileostomies, complications include irritant or allergic contact dermatitis and peristomal ulcers. 44 The latter condition can be caused by pressure from a poorly fitting device, fistulae to the anterior abdominal wall, peristomal pyoderma gangrenosum (PG), or infected hematomas.…”

Skin Manifestations of Inflammatory Bowel Disease

“…For more severe or refractory orofacial CD, treatment should include systemic steroids, immunomodulators, and anti-TNF-α therapy (Quezada et al, 2009). …”