Diagnostic and Management Challenges Associated With Vulvar Crohnʼs Disease

Abstract

Crohn's disease(CD) is an IBD with extra intestinal manifestations such as arthritis, uveitis, erythema nodosum. Crohn's disease of the vulva is a rare and under recognized condition, particularly because it may not involve GI tract. The diagnosis and treatment of this condition remains a challenge. We present a case of Vulvar Crohn's which has been an extremely difficult case to manage. Our patient is a 29 y/o female with 10 year h/o vulvar Crohn's disease presenting as painful fissures, perirectal and labial masses. On physical exam, there was erythema, induration and severe edema of labia majora, minora and perianal area. She underwent biopsy of the lesions which was confirmed to be Crohn's disease. Even though she had no GI symptoms, she underwent colonoscopy to determine the extent of her disease and it was normal. Patient was tried on several medications including metronidazole, steroids, 6-MP, infliximab, adalimumab, methotrexate, azathioprine, certolizumab,ustekinumab and vedolizumab. However, she failed to respond to any of these therapies. Patient was referred to plastic surgery for partial vulvectomy, biopsy of which showed high grade vulvar intraepithelial neoplasia (VIN) in addition to Crohn's (dermal chronic inflammation with granulomas). She was then referred to colorectal surgery for management of perianal disease, and gynecology for evaluation of VIN. She also underwent excision of anal skin tags and anal mapping, with pathology demonstrating positive P16 in squamous epithelium consistent with high grade AIN. Vulvar Crohn's is an under-recognized condition with about 100 cases reported to date. It can occur by direct extension from perianal region or by metastatic spread from bowel. Vulvar CD may precede intestinal involvement in about 25% of cases. It usually presents with edema, ulcerations and fissures of the vulva. Patients may be asymptomatic or present with pain, pruritis, discharge and dyspareunia. Diagnosis requires a high degree of suspicion as it could be mistaken for other conditions like infections, sarcoidosis etc. Diagnosis is confirmed by biopsy. Treatment remains a challenge and no treatment guidelines exist. A multidisciplinary approach is recommended and medications including steroids, thiopurines, TNF-α antagonists could be used. However, patients may not respond to any medications and surgery remains an option. Clinicians must be astute to consider neoplasia in this situation, as was seen in our patient.Figure: Microscopic findings of the epiglottis biopsy demonstrating in the high- and low-power fields ulcerated squamous mucosa with chronic inflammatory changes.