A Case Report of a Patient with Crohnʼs Disease Complicated by Pyoderma Gangrenosum Treated with Intravenous Immunoglobulin

Abstract

Purpose: We report a case of a 45-year-old male with a history of ileal, fistualizing Crohn's disease. The patient was originally diagnosed with ulcerative colitis status post ileal-pouch anal anastomosis that was reversed. He later developed severe perianal disease and his disease process appeared consistent with Crohn's disease (CD). He had multiple ileostomies with multiple complications most significant of which was severe difficult to control peristomal pyoderma gangrenosum (PG). The patient initially was treated with infliximab (Remicade®), but experienced an infusion reaction. He was then switched to adalimumab (Humira®) with mild benefit, but continued to have severe PG. He was later treated with anakinra without significant effects. His PG remained severe and required long-term steroids. He was later treated with cyclosporine with mild improvement. This had to be stopped in the setting of renal failure. He was also treated with methotrexate, which significantly worsened his symptoms. He was switched to mycophenolate mofetil (CellCept) which was also stopped in the setting of renal failure. Eventually, the patient was started on intravenous immunoglobulin (IVIG) which resulted in dramatic improvement to his PG as well as his CD. One year later, the patient has been off of adalimumab, continuing treatment with only IVIG, on minimal dose of steroids and has been feeling very well with complete clearance of his PG. Pyoderma gangrenosum (PG) is a rare, inflammatory neutrophilic dermatosis of unknown etiology. It is the second more frequent and most debilitating cutaneous extra intestinal manifestation in inflammatory bowel disease (IBD) patients. It is commonly treated with immunosuppressants and systemic corticosteroids; however, patients have reported that long-term usage of these medications cause serious side effects. Although there are only a few studies available, a very effective and safe therapeutic alternative for PG has been high dose IVIG with minimal side effects. IVIG has also been used in patients with Crohn's disease with great response. With limited data about the use of IVIG in Crohn's patients, the exact dose and duration of treatment remain to be defined.