Primary Chylous Lymphangiectasia of Female Genitalia

We report a case of a 50-year-old female who presented with multiple thick-walled vesicles involving the labia majora and minora which started developing at the age of 10 year. The vesicles extruded milky white fluid when punctured. Biochemical analysis of the fluid was consistent with chyle. Lymphoscintigraphy showed reflux of the radioactive tracer into the genitalia, thus confirming the diagnosis of primary chylous lymphangiectasia. The patient was not willing to undergo definitive surgical treatment. We report the case considering the rarity and to depict the usage of biochemical analysis of the vesicular content and lymphoscintigraphy to detect this entity.