S4939 Concomitant Cutaneous Crohn’s Disease and Pyoderma Gangrenosum Without Intestinal Involvement Treated With Guselkumab and Antimicrobial Agents

Introduction: Cutaneous Crohn’s disease (CD) is a rare manifestation of inflammatory bowel disease characterized by cutaneous lesions that are non-contiguous with gastrointestinal tissue yet share similar histopathologic features with gastrointestinal CD. Conversely, well-described extraintestinal manifestations of CD such as pyoderma gangrenosum (PG) are sequela of luminal inflammation with distinct pathology from that of CD. We present a case of concomitant cutaneous CD and PG without intestinal involvement. Case Description/Methods: A 64-year-old woman with perianal fistulizing CD (s/p total colectomy, completion proctectomy, and end ileostomy) and not on maintenance therapy presented for 2 months of worsening wounds in the groin, gluteal cleft, and peristomal skin. She was diagnosed with CD at age 60 years with severe perianal disease with local fistulization and perianal abscesses but no luminal disease. She was treated with infliximab, adalimumab, vedolizumab and upadacitinib and developed antibodies to the anti-tumor necrosis factor and disease progression with all of these therapies. She subsequently had staged proctocolectomy and was off all treatments. At 6 months post-operatively an ileoscopy confirmed no endoscopic or histologic inflammation. She presented to us 1.5 years later with new linear, knife-like ulcerations in the groin and gluteal cleft and peristomal ulcerations with gunmetal gray borders. Punch biopsy of a gluteal ulcer revealed scattered epithelioid granulomas within the dermis consistent with cutaneous CD. Ziehl-Neelsen and Fite stain were both negative for mycobacteria. The peristomal lesions had focal cribriform scarring clinically consistent with PG. During her hospitalization she received oral prednisone and topical steroids, and she was discharged on guselkumab, a steroid taper, and empiric antimicrobial therapy with clarithromycin, rifabutin, and ciprofloxacin. At 6-week follow up, she had significant improvement in pain, drainage, and size of both her gluteal and peristomal wounds. Discussion: In the absence of luminal disease, cutaneous CD and PG have important implications for treatment and patient quality of life. The management of cutaneous CD is described in case reports with antimicrobials, immune modulators, and biologic agents without standardized treatment algorithms. This is a novel case of concomitant PG and cutaneous CD treated with a novel combination of guselkumab and combination antimicrobial agents.