Congenital Vulvar Lymphangioma (Lymphoedema) Circumscriptum in a Young Female

Abstract

Lymphangioma circumscriptum (LC) is a form of lymphangioma characterized by benign dilation of lymphatic channels, which affects the skin and subcutaneous tissues. The most common sites are mucosa of mouth, tongue, groin, axilla, trunk and proximal region of extremities. Vulva is a rare site of LC. These abnormal lymphatic malformations do not communicate to the normal lymphatics. A female patient, aged 17 years, presented with papule-like condyloma of the labia majora. After simple vulvectomy was performed, symptoms improved. Histology revealed lymphangioma circumscriptum of the vulva. LC is a rare benign pathology that should be kept in mind in any vulval condylomatous lesion. Till date, only a few cases of LC of vulva have been reported in the literature. The treatment of primary vulvar lymphangioma circumscriptum is mainly surgery. Depending on the age of the patient and the extent of lesion, surgical treatment might be in the form of the local excision, labiectomy, or vulvectomy.