S2252 Vulvar Involvement in Crohn’s Disease Successfully Treated With Ustekinumab

Abstract

INTRODUCTION: Crohn’s Disease (CD) can involve tissues outside the gastrointestinal tract including, rarely, the skin. Vulvar cutaneous involvement is uncommon, affecting around 2 % of female patients. Since the rash is atypical, a biopsy is needed to confirm the diagnosis. Physicians should be vigilant about this condition and early intervention is needed to prevent further complications. CASE DESCRIPTION/METHODS: We are reporting the case of a 22-year woman diagnosed with small bowel CD at the age of 11. Over time she developed a stricturing phenotype (B2). She had previously been treated with anti-TNF agents and was currently on maintenance therapy with vedolizumab and methotrexate with a modest clinical and endoscopic response. She reported swelling of her right labia for several weeks associated with mild discomfort. On examination, she had erythema and gross thickening of the vulvar skin with some desquamation but no drainage or fluctuance. Laboratory workup was unremarkable except anemia (Hb 7.1g/dL). CRP was 0.6 mg/dL (0-1 mg/dL). She had active inflammation and a stricture in the terminal ileum on CT Enterography. Pelvis MRI showed no peri-anal disease. At colonoscopy, she had moderate inflammation in the terminal ileum and two strictures which required dilation. Due to persistent symptoms and lack of response to therapy the patient underwent an ileocolic resection. A labial skin biopsy was obtained intraoperatively, which revealed perivascular chronic inflammation, focal non-necrotizing granuloma, and intralymphatic histiocytes consistent with CD (Figure 1, 2). Postoperatively she was switched to ustekinumab and methotrexate. She recovered very well, and at 3 and 9 months follow up she reported substantial improvement in both her luminal symptoms and her peri-vulvar disease. DISCUSSION: Vulvar involvement is a rare and often difficult to diagnose cutaneous manifestation of CD. Peri-vulvar CD can occur as a contiguous spread of perianal disease or it can be a “metastatic” isolated involvement. Vulvar erythema and edema are the most common findings which can cause scarring and significant deformity. Therapeutic options include antibiotics and topical steroids for mild cases or systemic immunosuppressives for more severe forms. To our knowledge, this is the first report describing a patient with vulvar cutaneous involvement from CD who was successfully treated with ustekinumab after failing vedolizumab. Further research with this therapeutic agent for cutaneous CD is warranted.Figure 1.: Low power (×40) view of labial biopsy.Figure 2.: High power (×200) view of labial biopsy showing non necrotizing granuloma and focal giant cell reaction.