A newly described possible complication of lymphangioma circumscriptum

Lymphangioma circumscriptum is a rare disorder of lymphatic channels, the cause of which is poorly understood. It is an unpleasant, but benign, condition conventionally managed by wide local excision, which provides symptom control and often prevents recurrence. No long term complications and no association with squamous cell carcinoma have been previously documented. We present a 20-year-old patient with lymphangioma circumscriptum involving the vulval and perianal areas, which recurred after surgery and seems to have predisposed to squamous cell carcinoma 11 years after presentation.