Vulvar High‐Grade Squamous Intraepithelial Lesion in Primary Lymphedema With Severe Genital Lymphorrhea: A Case Report

Vulvar high-grade squamous intraepithelial lesion (HSIL) is an HPV-associated premalignant lesion requiring prompt management. Its occurrence in primary lymphedema is rarely described. We report a 37-year-old woman with childhood-onset primary lymphedema who presented after 31 years of clinical quiescence with recurrent right lower-extremity and genital lymphedema presenting with vulvar swelling, erythema, follicular lesions, and persistent lymphorrhea. Indocyanine green (ICG) lymphography confirmed recurrent combined genital and lower-extremity lymphedema. Lymphaticovenular anastomosis (LVA) and abnormal perineal tissue excision were performed during the same operative session at different sites. Histopathology unexpectedly demonstrated multifocal vulvar HSIL with lymphangiectatic change. Immunohistochemistry showed p16 and Ki-67 overexpression, wild-type p53, D2-40 positivity, and CD34 negativity. HPV genotyping detected HPV16 and HPV42. Lymphorrhea decreased substantially, and leg volume decreased by 9.5%. Chronic genital lymphedema can obscure premalignant disease; morphologically abnormal or persistent vulvar lesions warrant histopathological assessment. LVA may reduce lymphorrhea and edema but cannot replace definitive oncologic treatment.