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159 REFRACTORY CROHN'S DISEASE OF THE VULVA (MENDELSOHN-ROSENTHAL SYNDROME) TREATED WITH INFLIXIMAB: A CASE REPORT S Makhija, S Coderre, R Panacionne Crohn's disease (CD) is a chronic inflammatory disorder primarily involving the intestine. Metastatic Crohn's disease is a granulomatous extraintestinal manifestation of CD with varied presentations. Approximately one-quarter of Crohn's patients can present with gynecological complications, such as chronic induration of the vulva and fistulas to the vulva and vagina. The lack of awareness and delay in diagnosis can lead to more severe complications.
University of Calgary, Calgary, Alberta
CASE: We present the case of a 45-year-old woman diagnosed with CD at the time of laparotomy for free perforation of the sigmoid colon. She was placed on postoperative 5-ASA. Three years postoperatively, she developed progressive swelling of the right and left labia. There was no evidence of a rectovaginal fistula on gynecological exam. Vulvar biopsies demonstrated granulomas consistent with Crohn's disease of the vulva, also known as Mendelsohn-Rosenthal syndrome. Initial treatment consisted of metronidazole without benefit. She was later placed on high-dose corticosteroids and subsequently azathioprine 2.5 mg/kg without appreciable improvement of the labial swelling and induration. The patient was started on infliximab 5 mg/kg at zero, two and six weeks and continued the azathioprine. There was improvement in labial swelling with the initial induction infusions and then was placed on maintenance episodic infliximab therapy. Repeat labial biopsies failed to show any residual granulomas.
CONCLUSIONS: Metastatic Crohn's disease to the vulva is a rare presentation. We report a case refractory to standard therapy, which resolved clinically and histologically on infliximab. This is the first known reported case in the literature.