INTRODUCTION
Extramammary Paget Disease (EMPD) is a rare intraepithelial malignancy of pluripotent keratinocyte stem cells that presents on apocrine-rich skin of the perineum, vulva, and less commonly, axilla.1 EMPD clinically presents as a slow growing, unilateral, strawberry-pink scaly patch or plaque, frequently impacting Caucasian women in their sixth to eight decades (Figure 1).1,2
apoptosis.2,3 Imiquimod can be used as monotherapy, adjunctive therapy before or after surgery, as well as part of a therapeutic combination with other management modalities. Complete remission (CR) when used as a single agent ranged from 52% to 72%, according to one study.2,3 Eighty-five percent of patients experienced greater than 50% clinical regression; unfortunately,
While typically confined to the epidermis, EPDM can be invasive, associated with contiguous extension or upward pagetoid spread of underlying neoplasms or with distant synchronous malignancy.3 The complexity of EMPD intertwined with the heterogeneity of the disease in its appearance, location, and depth of invasion, often requires a multidisciplinary approach to management (Table 1).1
There have been recent significant developments in further characterizing EMPD, such as identification of associated mutations in TP53, ERBB, NRAS, BRAF, PIK3CA, and AKT1 genes and overexpression of P16 protein and the HER2 and Androgen Receptor (AR) signaling pathways.2 However, given EMPD is a rare disease, there are no established guidelines regarding diagnosis and treatment modalities.2,3,4 Herein we review evidence and provide insight for non-surgical and surgical approaches utilized for EMPD.
Non-surgical Management
EMPD often elicits inherent surgical limitations due to its aggressive nature, ill-defined margins, and subclinical extension; therefore, conservative treatment approaches are ideal.5,6
Imiquimod
As a toll-like receptor 7 agonist, imiquimod induces innate and cell-mediated inflammatory responses and subsequent cell
apoptosis.2,3 Imiquimod can be used as monotherapy, adjunctive therapy before or after surgery, as well as part of a therapeutic combination with other management modalities. Complete remission (CR) when used as a single agent ranged from 52% to 72%, according to one study.2,3 Eighty-five percent of patients experienced greater than 50% clinical regression; unfortunately,
