Mammary and extramammary Paget's disease

Abstract

Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.

FIGURE 1

A: Initial mammary Paget’s diseas. Observe unilateral involvement on right nipple. B - Detailed depiction on the previous figure (Courtesy from Dr. Samuel Freire da Silva) C- Mammary Paget’s disease also affecting the areola

FIGURE 2

Mammary Paget’s disease in the nipple associated, in the same biopsy, with mammary duct in situ carcinoma (HE, 40x)

FIGURE 3

Vulvar Paget’s disease

FIGURE 4

Paget’s disease affecting vulva, perineal region and part of the inguinocrural sulcus

FIGURE 5A AND 5B

Extramammary Paget’s disease in male genitals

FIGURE 6

A: Epidermis showing intense thickening due to the proliferation of atypical cells in Paget’s disease (HE, 40x). B: Pagetoid migration of atypical epithelial cells, near the granular layer, some with a clear cytoplasm (HE, 400x)

FIGURE 7

CK7 immunoreactivity in several neoplastic cells (400x)

FIGURE 8

BRST2 or GCDFP-15 m a r k e r (gross cystic disease fluid protein-15) with focal positivity in E M P D (400X)