Mammary and extramammary Paget's disease

Abstract

Paget's disease is an intra-epidermal adenocarcinoma seen over the nipple/areola (mammary Paget's disease) or in extramammary body zones, such as the anogenital and perineal skin and the axilla. Mammary and extramammary Paget's disease share many common clinicopathological features but also show several differences, namely, with regard to pathogenesis and association with underlying malignancies. Indeed, mammary Paget's disease is as a rule associated with an underlying breast carcinoma whereas association of extramammary Paget's disease with underlying (skin or visceral) malignancies occurs much less frequently. We review here the main clinicopathological and therapeutic features of mammary and extramammary Paget's disease.

Paget’s disease of the breast (a review of literature)

“…2б, в). В 15-25 % случаев пациенты предъявляют жалобы на боль, жже-ние и зуд в области соска [27]. РП обычно носит од-носторонний характер, но были отмечены и случаи поражения обеих МЖ [28,29].…”

The case of an extensive primary extramammary Paget's disease diagnosis and treatment

“…Primary EMPD originate from intraepidermal cells. Secondary EMPD is associated with underlying malignancy and accounts for 10-30% of all EMPD cases series [1,5,6]. The most common associated neoplasms arise from the bladder, urethra, prostate and rectum.…”

“…Cutaneous Paget's disease is a rare intraepithelial neoplastic condition [1,2]. The breast Paget's disease can be associated with invasive and in situ breast cancer and is a well-defined entity.…”

Cutaneous manifestations of cancer and chemotherapy

“…3 Serological testing (ELISA and Western blot) is sensitive, but not specific; both tests may be negative early in the disease and they do not accurately distinguish active from past infection. Lyme disease can be confirmed only by culture, although this is a low yield procedure.…”

“…Paget's disease is an intra-epidermal carcinoma seen in the nipple and areola area (mammary Paget's) or anogenital and axillary skin (extramammary Paget's). 3 Paget's disease of the breast presents as unilateral, sharply demarcated nipple eczema or erosion, often with bleeding or nipple discharge, in association with underlying intraductal adenocarcinoma of the breast or non-invasive carcinoma. Extramammary Paget's disease is characterised by an itchy, moist, well-defined erythematous plaque, typically in the anogenital area and most common in elderly women.…”