Abstract
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis strongly associated with inflammatory bowel disease (IBD). This narrative review summarizes current knowledge on the epidemiology, clinical features, proposed mechanisms, and treatment of PG in patients with IBD. In addition to population-based, large cohort, and mechanistic studies, we reviewed 115 published case reports and case series describing patients with PG and IBD and synthesized demographic, clinical, and therapeutic trends. Most patients developed PG after an IBD diagnosis, with smaller proportions presenting simultaneously or before an IBD diagnosis. PG in IBD patients typically affects middle-aged adults and has a female predominance. Clinical features are heterogeneous, which complicates recognition and timely diagnosis. Treatment responses are also highly variable. Corticosteroids and immunosuppressants are commonly used as first-line therapies, but many patients require sequential or combined regimens. Biologics are increasingly used, reflecting efforts to target shared inflammatory pathways between PG and IBD; however, treatment approaches remain highly individualized. Mechanistic and genetic studies implicate Th17/Th1 immune dysregulation, IL-1β/IL-36 signaling, and gut-skin immune crosstalk. There is a critical need for longitudinal, controlled studies to clarify pathogenesis, predict outcomes, and guide evidence-based, standardized treatment approaches in this complex patient population.