Observational study of metastatic vulvar Crohn's disease in the pediatric population

Objective: Metastatic vulvar Crohn's disease (MVCD) is a rare cutaneous manifestation of Crohn's disease (CD), especially in pediatric patients. The objective of this study is to assess presentation, diagnosis, and treatment for pediatric MVCD in the largest cohort reported. Methods: This is a retrospective review of pediatric patients with MVCD from 2014 to 2024. Results: Twelve patients with MVCD were identified ranging from 7 to 17 years old at time of diagnosis. On average, MVCD was diagnosed 3 years after CD. Most were diagnosed with MVCD after CD but two were diagnosed concurrently and two had vulvar symptoms prior to gastrointestinal (GI). All patients were diagnosed clinically with only one patient undergoing skin biopsy (8.3%). Most common signs of MVCD included vulvar edema and linear fissures. Ninety-two percent of patients had an abdominal and pelvic magnetic resonance enterography (MRE), all with findings concerning for MVCD. All patients were treated with biologics as the main modality, along with adjunctive antibiotics (83%) and topical steroids (50%). Only 42% had documented remission of vulvar disease as determined by patient-reported symptom resolution. Conclusion: Physical exam findings and MRE are essential in diagnosis of MVCD in the pediatric population. Standardized treatment guidelines are lacking, and more than half of our patients did not achieve resolution of their vulvar symptoms even with well-controlled GI disease. The need for further research and investigation into optimal management strategies is essential.