2048 A Rare Manifestation of a Not so Rare Disease

Abstract

INTRODUCTION: Pyostomatitis vegetans (PV) is a rare mucocutaneous disease characterized by miliary pustules and ulcerations affecting mucous membranes. PV is classified as a subtype of pyoderma gangrenosum, which affects oral mucosa. The majority of PV cases are associated with inflammatory bowel disease. We present an interesting and a rare case of PV associated with ulcerative colitis (UC) that was successfully treated with infliximab. CASE DESCRIPTION/METHODS: A 60-year-old female with history of ulcerative proctitis who had been off therapy for one year presented with abdominal pain, bloody diarrhea, painful oral ulcers, and a 25-pound weight loss over one month. She first noticed the oral ulcers two weeks prior to presentation, and they worsened to a point that she could not eat. Physical examination revealed confluent ulcerations of the buccal mucosa and diffuse abdominal tenderness. Blood work showed hemoglobin of 8 gm/dl, albumin of 2.5 gm/dl, ESR of 101 mm/hr, and CRP of 17 mg/L. C diff was negative. Oral lesions were negative for HSV-1 and HSV-2 by PCR. CT-abdomen showed findings consistent with active left sided colitis. The patient was started on PO and rectal mesalamine and IV methylprednisolone. Colonoscopy revealed severe deep ulcers extending from anus to proximal sigmoid colon with spontaneous bleeding and complete loss of vascular pattern (Figure 1a). During EGD large confluent ulcerations on bilateral buccal mucosa were visualized (Figure 2a–c). Biopsy of the oral lesions revealed ulcerated squamous mucosa and underlying skeletal muscle with mixed, predominantly chronic inflammation. Immunostains for HSV and CMV were negative. Due to minimal response to IV steroids, patient was started on infliximab 10 mg/kg, resulting in dramatic improvement of oral lesions and GI symptoms. Azathioprine was added for dual therapy for the first year. Endoscopic and clinical remission of colitis was achieved (Figure 1b) in addition to complete resolution of PV (Figure 2d). Patient continues to be in remission on infliximab monotherapy two years from initial presentation. DISCUSSION: To the best of our knowledge, this is the second reported case of UC with PV successfully treated with infliximab. Our unique case demonstrates a rare extraintestinal manifestation of UC, namely PV, that highlights the importance of early and aggressive anti-TNFα therapy to achieve remission of PV and colitis.