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.

Paget’s disease of the breast: study of cases series

“…The invasion of attachment structures may occur. The dermis presents reactive characteristics, such as telangiectasia, chronic inflammation, and ulceration in advanced cases 6 . In our series, the most common differentiation degree was "moderate or high" (83.33%) and the most frequent histological type was "in situ ductal carcinoma" (66.7%).…”

Extramammary Paget Disease: Case Report

“…Sıklıkla postmenppozal kadınlarda, ekzematöz, eritemli, skuamlı lezyon olarak görülür. 1 Ekstramammaryan Paget hastalığı (EMPH) ise daha nadir olarak görü-lür ve mammaryan türünde olduğu gibi genellikle postmenopozal kadınları etkiler. 1 Sıklıkla etkilenen bölgeler vulva, sonrasında perineal, perianal, skrotal bölgelerdir.…”

“…1 Ekstramammaryan Paget hastalığı (EMPH) ise daha nadir olarak görü-lür ve mammaryan türünde olduğu gibi genellikle postmenopozal kadınları etkiler. 1 Sıklıkla etkilenen bölgeler vulva, sonrasında perineal, perianal, skrotal bölgelerdir. 2 Vulvar EMPH, vulvar malignitelerin %1-5'ini oluştu-rur.…”

“…1 Histopatolojik olarak ayırıcı tanısında ise pagetoid Bowen hastalığı, pagetoid in situ malign melanom yer alır. 1 Tedavisinde geniş cerrahi eksizyon tercih edilir. Cerrahi eksizyonun prognozu iyidir, rekürrens %30-60 oranında görülür.…”

“…6 Radyoterapi, 5-florourasille medikal tedavi, bleomisin, CO 2 lazer, topikal imiquimod tedavileri uygulanan diğer yöntemler-dir. 1 Topikal tedavi birkaç vakada etkili olmakla birlikte, %50 oranında relaps görülmüştür. İlişkili olan bir internal malignite veya dermal, lenfositik infiltratta CEA pozitifliği varsa prognoz daha kö-tüdür.…”