IVIG Treatment for Refractory Pyoderma Gangrenosum in Patients with Inflammatory Bowel Disease

Abstract

Purpose: Pyoderma gangrenosum (PG) is a chronic, immune-mediated skin disorder characterized by ulcerating skin lesions. It can have devastating consequences including pain, disfigurement, decreased quality of life, and even death. Most patients have an underlying systemic disease such as inflammatory bowel disease (IBD). Pathogenesis is unclear, and there is no gold standard for treatment. Different topical and systemic therapies have been tried with limited results. We present two cases with IBD-associated refractory PG who have failed multiple therapies. Case 1 is a 44-year-old white male who was diagnosed with left-sided ulcerative colitis (UC) at age 12. He developed PG at age 29 that has involved both his right and left ankles. Physical exam shows a 2 × 2 cm PG lesion on the left medial malleolus with drainage and erythema. His treatment has included steroids, calcineurin inhibitors, thiopurines, infliximab, and surgery (prior skin grafting and venous ablation). He has also been treated with IV antibiotics for secondary infection. He had open leg ulcers that were painful and draining fluid interfering with his ability to work. It was decided to pursue treatment with intravenous immunoglobulin (IVIG) given the severity of the disease. In the hospital, he received 3 doses of 1 mg/kg on consecutive days, and he tolerated them well. On follow up exam one week later, he had significant improvement in ulcer size, pain, and mobility. Case 2 is a 23-year-old white female who was diagnosed at age 8 with Crohn's disease (CD) and has had a complicated course requiring a distal ileal resection now with an end left colostomy. She has been unable to consider surgery due to persistent biopsy proven perianal PG. Physical exam shows for a 1 × 1 cm ulcerated area near the gluteal folds with tenderness and erythema. Her treatment has included methotrexate, steroids, infliximab, natalizumab, adalimumab, and tacrolimus. She has had multiple hospital admissions for pain management. She received IVIG 1 mg/kg for 3 consecutive days monthly for three months. Her PG lesions have significantly healed, and she has returned to college. Treating PG is challenging for gastroenterologists. Biologic therapy has been shown to be an effective and safe treatment for PG in patients with IBD, but many patients have refractory lesions with limited options. IVIG is more often being used to treat inflammatory and autoimmune diseases to target defective cell-mediated immunity. We report two patients with refractory IBD-associated PG treated with IVIG who both had significant improvement in their respective wounds and overall quality of life. Given the effectiveness and safety profile, IVIG should be considered as second-line therapy.