Vulvar Crohn’s Disease Progression Over 20 Years: A Case Study




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Background: Vulvar Crohn’s disease (VCD) is a rare cutaneous manifestation of general Crohn’s disease (GCD), characterized by non-caseating granulomas in the vulvar region. VCD occurs independently of fistulization, and in 25% of cases, the vulvar manifestation precedes gastrointestinal symptoms. This case presentation describes the disease course in a patient followed longitudinally over 2 decades. The case report aims to provide insights into the presentation, diagnosis, and management of VCD, as well as highlight the challenges and considerations in clinical practice. Case information: An 18-year-old female initially presented with a two-week history of worsening pain in her vulvar area. (See Figure 1.) She had vaginal discharge and noticed multiple open lesions in the vulvar area. She was sexually active. The patient denied any gastrointestinal symptoms such as abdominal pain, diarrhea, or bloody stool. Primary differentials on initial presentation included donovanosis, lymphogranuloma venereum, hidradenitis suppurative, HIV, and cancer. Biopsy of the vulvar lesions revealed non-caseating granulomas, which are consistent with Crohn’s disease as depicted in Figure 2. The ulcerations spontaneously resolved one year after initial diagnosis as shown in Figure 3. Over the next 20 years, the patient had progressive disease despite use of immunosuppressive agents. The disease has now progressed to a rectovaginal fistula and complete obliteration of the vulvovaginal and anal architecture. (See Figure 4.) Management challenges for this patient included intermittent access to care due to socioeconomic factors and suboptimal response to immunosuppressive therapy. Conclusion: There are fewer than 300 cases of VCD reported in literature. VCD presents a unique diagnostic challenge, often confused with infectious etiologies. The differential of VCD should be considered in patients with vulvar fissures and edema. Management of VCD is more intricate than diagnosis and is not well understood. The literature shows that intestinal disease may be well controlled with a TNF-inhibitor, but VCD may continue to flare. A multidisciplinary approach involving multiple specialists is essential for optimal management. This topic requires further research on therapeutic approaches to improve patient outcomes.