Vulvar Crohn’s Disease Manifesting as Vulvar and Perianal Fissuring

Summary: A woman in her 40s presented with chronic vulvar and perianal fissures extending from the anterior anus to the perineal body and introitus. Examination revealed diffuse swelling, induration, fissuring, and superficial erosions in cobblestone appearance across the vulvar and perianal skin. Biopsies demonstrated dense lymphoplasmacytic and histiocytic dermal inflammation with scattered neutrophils and eosinophils and areas of scarring and granulation tissue. EGD and colonoscopy were unremarkable. MR enterography showed vulvar and perianal skin thickening and hyperenhancement consistent with granulation tissue and fissures. Findings were consistent with cutaneous Crohn’s disease. Vulvar Crohn’s disease is a rare extraintestinal manifestation of Crohn’s disease presenting with chronic edema, fissures, or ulcerations. Lesions often present independently or prior to active gastrointestinal disease and may lead to delayed diagnosis. The pathophysiology likely involves localized immune dysregulation leading to granulomatous inflammation. Dermatopathology typically reveals non-caseating granulomas and chronic inflammation, though findings may vary, with one cohort with only 38% of patients having the classic noncaseating granulomas on biopsy. Management strategies involve systemic treatments such as corticosteroids, immunomodulators, or TNF inhibitors. In refractory cases, surgical intervention may be considered. Early recognition and interdisciplinary care are essential to improve outcomes and quality of life.