Implications of Adnexal Invasions in Primary Extramammary Paget’s Disease: A Systematic Review

Abstract

Extramammary Paget’s disease (EMPD) is an erratic malignant skin disorder primarily affecting skin areas abundant with skin appendages like hair follicles. The vulva is most involved site, followed by genital areas, penoscrotal regions and axillary skin. EMPD presents as erythematous skin lesions resembling eczema, typically progressing slowly, either primary or secondary manifestations. Primary EMPD originates as an intraepithelial neoplasm of the epidermis, often leading to local lymph node metastases and distant metastases. A systematic literature search using targeted keywords across multiple databases was conducted. Studies focusing on EMPD, adnexal involvement, depth, recurrence, and prognosis were included by keeping in view the objective which is to determine the significance of adnexal involvement and depth concerning recurrence and prognosis in the primary EMPD. Adnexal involvement, especially in hair follicles and eccrine ducts, is prevalent in primary EMPD. However, its correlation with tumor progression or recurrence rates remains inconclusive. Surgical excision, including Mohs micrographic surgery, is the primary therapeutic approach, with topical agents and systemic treatments used in advanced cases. Future studies regarding understanding adnexal involvement's depth and significance are essential in designing effective targeted therapeutic approaches in EMPD.