Isolated Oral Pyoderma Gangrenosum Following Proctocolectomy

Abstract

Purpose: A 20-year-old man with a 6 month history of ulcerative colitis (UC) presented with a severe flare requiring corticosteroids and infliximab. He presented 3 months later with worsening symptoms of bloody diarrhea and fever despite escalating doses of infliximab. After two weeks of intensive treatment, he ultimately underwent a proctocolectomy. In the postoperative period, steroids were tapered and he gradually developed severe odynophagia and mouth pain. Examination revealed what appeared to be angular cheilitis and aphthous ulcers on the tongue, uvula and posterior oropharynx (Figure 1). No skin lesions were noted on examination. The oral lesions were biopsied, given their suboptimal response to conservative management, demonstrating squamous mucosa with acute inflammation and ulceration. The underlying soft tissue showed diffuse granulomatous inflammation characterized by epithelioid histiocytes with focal giant cells surrounding central neutrophilic infiltrates with leukocytoclasia (Figure 2). No vasculitis or viral inclusions were present. Special stains for fungi (GMS) and acid-fast bacilli (AFB) were negative. A diagnosis of PG was established and confirmed by an expert pathologist. The patient was given high-dose intravenous corticosteroids and he began to show improvement in symptoms as well as ulcer healing by three days. Pyoderma Gangrenosum (PG) is a primarily dermatologic manifestation of inflammatory bowel disease seen in 1-12% of patients with UC and less commonly in Crohn's colitis. Fewer than 10 cases of stomal PG have been reported and we describe a unique case of isolated PG of the oral cavity in a post-colectomy patient with UC.Figure: No Caption available.Figure: [1305]