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| Fig. 61.4 Genital extramammary Paget’s disease demonstrating marked erythema with superficial erosions. (Courtesy of Whitney A. High, MD.) |
Paget’s disease (Fig. 61-4) is a neoplasia of the vulva that consists of adenocarcinoma-type cells that invade the epidermis, the appendages of the skin, and occasionally the dermis. It represents 1% of vulvar neoplasms. However, 25% of the time that extramammary Paget’s disease is present, it is associated with an underlying adenocarcinoma (most commonly of the skin adnexa or Bartholin gland) or at a distant site (e.g., the breast, genitourinary system, or the intestinal tract). Thus, it is prudent to search for other possible malignancies if Paget’s disease of the vulva is diagnosed.
Shaco-Levy R, Bean SM, Vollmer RT, et al: Paget disease of the vulva: a histologic study of 56 cases correlating pathologic features and disease course,
Int J Gynecol Pathol 29:69–78, 2010.
