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Crohn's disease (CD) is a multi-systemic chronic granulomatous inflammatory disease with a constellation of extraintestinal manifestations affecting various organs, including the skin. Metastatic CD (MCD), defined as granulomatous lesions of the skin occurring at sites separate from the gastrointestinal (GI) tract in patients affected with CD, is the least common cutaneous manifestation, and it is occasionally the primary manifestation of the disease. It may precede any symptoms from the GI tract by months or even years, and it often remains undiagnosed. We present the case of a 42-year-old woman with a rare cutaneous manifestation of MCD involving the external genitalia. The cutaneous lesions of the mons pubis and vulva preceded GI manifestations of CD by 2 years. The patient was treated with azathioprine, oral steroids, and antibiotics. Reduction of the cutaneous nodules, discharge, and erythema was achieved, accompanied by relief of GI symptoms. Response to treatment was maintained during a follow-up of 6 months. In MCD, involvement of the external genitalia is exceedingly rare, with few documented cases in the literature. The integration of clinical information, microscopic findings, and exclusion of other cutaneous granulomatous processes is necessary in order to accurately diagnose this rare dermatologic entity and provide an opportunity for successful therapeutic intervention.

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