Can vulvar edema be the presenting sign of Crohn's disease? A case series

Vulvar involvement as an extraintestinal manifestation of Crohn's disease (CD) is a challenging diagnosis considering that vaginal findings may precede gastrointestinal symptoms. The aim of this study is to describe the clinical presentation, radiological findings, and treatment of vulvar Crohn's disease (VCD). We reviewed the time from initial presentation to diagnosis, presenting symptoms, radiological findings, gastrointestinal Crohn's disease Paris classification, and treatment response of five female pediatric patients. All the patients had radiological findings of vulvar inflammation on magnetic resonance imaging. Vaginal symptoms preceded gastrointestinal disease in two of the patients, which correlated with a delay in diagnosis. All patients had active disease on colonoscopy, with three of them having significant colorectal inflammation. Four of the patients were treated with infliximab, while one patient received ustekinumab with a resolution of their symptoms. In conclusion, VCD can precede gastrointestinal symptoms and is easily miss-diagnosed, leading to a delayed Crohn's disease diagnosis and treatment. What is Known Symptoms of vulvar Crohn's disease (VCD) include labial swelling, pruritus, and vaginal pain. VCD can precede gastrointestinal involvement. Biopsies identify granulomas in less than 50% of cases of VCD. Treatment options include steroids, antibiotics, and biologics.What is New VCD as a presenting symptom can significantly delay diagnosis and treatment. Contrast-enhanced perineal magnetic resonance imaging may be considered a primary diagnostic tool to confirm VCD to avoid vulvar biopsies. Ustekinumab or high-dose infliximab can provide complete clinical resolution of vulvar symptoms.