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.