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A 56-year-old woman presented to the dermatologist for worsening vulvar papules and edema for 3 years. She had Crohn disease in remission status post colectomy with ileostomy and vaginal fistula repair. Prior nondiagnostic biopsies performed by the gynecology service showed squamous hyperplasia, lichenoid features, acute and chronic inflammation, and dermal fibrosis. The labia were markedly edematous, with clear to hemorrhagic papules and vesicles coalescing to a papillomatous plaque (Figure 1). These findings were suggestive of acquired lymphangioma circumscriptum (LC) secondary to Crohn disease. This diagnosis was confirmed by a skin biopsy, which showed cystically dilated vascular lymphatic spaces in the dermis (Figure 2). Clobetasol 0.05% cream daily, initiated by the gynecologist, resulted in significant improvement during several weeks (Supplemental Figure, available online at http://www.mayoclinicproceedings.org).
Lymphangioma circumscriptum, a benign lymphatic malformation, is most commonly seen in children on the trunk and extremities.
Treatment options include laser therapy, cryotherapy, sclerotherapy, surgical excision, and labiectomy. The lesions tend to recur. Rarely, lymphangiosarcoma arising in long-standing LC has been described.
This case is unique because treatment with a superpotent topical steroid led to clinical improvement, although a multimodal treatment approach will likely be required. Recognizing vulvar LC early is important to establish the correct diagnosis and management.