Metastatic vulvovaginal Crohn disease in the setting of well-controlled intestinal disease

The cutaneous manifestations of Crohn disease (CD) are varied and include pyoderma gangrenosum, erythema nodosum, and metastatic CD (MCD). The latter is defined as the occurrence of granulomatous lesions at a skin site distant from the gastrointestinal tract. Metastatic CD involving the vulva and perineum is rare and thus often is difficult to diagnose. It may precede, coincide with, or develop after the initial diagnosis of intestinal disease. A variety of clinical presentations have been described, including widespread nonspecific pain and swelling, erythematous papules and plaques, and nonhealing ulcers. The diagnosis often is delayed because of a low index of suspicion brought about by the rarity of the disease and its diverse and confusing manifestations. A skin biopsy usually confirms the diagnosis by revealing noncaseating granulomas in the dermis. Multiple oral and parenteral therapies are available, with surgical intervention reserved for resistant cases. We present a case of vulvovaginal MCD in the setting of well-controlled intestinal disease.