Lymphangioma Circumscriptum in Vulva with Klippel-Trenaunay Syndrome

To the Editor: Lymphangioma circumscriptum (LC) is not a tumor but rather a congenital malformation of the superficial lymphatics. It presents as groups of deep-seated, vesicle-like papules, resembling frog spawn, at birth or shortly thereafter. Also, the sites of predilection are the abdomen, axillae, genitalia, and mouth, particularly the tongue.[1] Klippel-Trenaunay syndrome (KTS) is relatively rare as a congenital vascular anomaly classically defined as the triad of vascular stain, venous varicosities, soft tissue, and/or bony hypertrophy. Many cases of KTS are mixed malformations with capillary, lymphatic, and venous elements.[2] Here, we reported a Chinese woman with vulvar LC coexisting with KTS in the same side of body.