Paget's disease is an eczematous skin change of the ' that is usually associated with an underlying breast malignancy.<unk>  was first discovered by James Paget, a British surgeon and physiologist, in 1874.The characteristic erythema and eczematous changes of the nipple seen with<unk>  were first described by Velpeau in 1856. The correlation between intraductal cancer and<unk>  was by Jacobeus in 1904.The background for the epidermotropic theory, that ducts containing carcinoma cells were apparently connected to overlying nipples containing Paget’s cells, was demonstrated by Muir and Inglis in 1939 and 1946 respectively. The first case of Paget's disease in a male was described by Elbogen in 1908..<unk>  occurs in 1 – 4% of all female breast carcinoma cases.<unk>  occurs in 0.5–5% of all breast cancer cases and is invariably associated with underlying malignancy either overt or occult. WHO  revealed that there are 800,000 up to 1 million new cases of breast cancer each year. An underlying breast carcinoma is found in >90% of patients with Paget's disease. The majority of these cases are invasive disease although 40 – 45% are associated with ductal carcinoma in situ.<unk>  is more prevalent in postmenopausal women, usually after the sixth decade of life, but it has also been reported in adolescent and elderly patients. Females are more commonly affected with<unk>  than males. Male breast cancer accounts for less than 1% of all breast cancer and Paget's disease represents 1-3% of all breast malignancies. Mammary Paget's disease is almost always associated with an underlying breast cancer in 92–100% of cases. Approximately 50% of this patients present with an associated palpable mass in the breast. On microscopic histopathological analysis, epidermal Paget cells which are malignant glandular epithelial cells organized in groups with nest-like patterns or gland-like structures and are preferably located in the epidermal basal layer are characteristic findings of<unk> . Common risk factors in the development of<unk>  are female more than male, incidence is higher with increase in age  and people of African or Ashkenazi Jewish descent. Personal or family history of breast cancer Environmental exposure to radiation, high temperature and electromagnetic fields. Genetic mutations like BRCA1, BRCA2 4-14% of male breast cancer, klinefelter's syndrome  in males. Mutations in RAD51B, PALB2, CYP17, CHEK2, BRIP1, RAD51C and androgen receptors. BRIP1 and RAD51C have not been seen in male patients. Endocrine-related states with hyperestrogenism such as obesity, exogenous estrogen, testicular dystrophic lesions in males. Symptoms of<unk>  include itching, redness, thickened skin, and ulceration of the nipple. Common physical examination findings of<unk>  include eczematous appearance of the nipple associated with yellowish or bloody discharge. Due to close similarity with many skin lesions, the diagnosis of Mammary Paget’s Diseas may be delayed or many cases can be misdiagnosed. Immunohistochemical staining for cytokeratin, epithelial membrane antigen (EMA) and c-erb-B2 oncoprotein is useful for the differential diagnosis. Toker cells found in the epidermis of the nipple, close to the opening of lactiferous ducts, along the basal layer of the epidermis, are morphologicaland immunohistochemical similar to mammary Paget's cells. In contrast to Paget's cells which are strongly associated with both Ki-67 and Her-2/c-erbB-2 and these markers are mostly used to distinguish Paget's cells from Toker cells. In case of atypical Toker cells a combination of CD138 and p53 is very helpful in distinguishing these atypical cells from Paget's cells.<unk>  must be differentiated from atopic dermatitis, eczema, psoriasis, malignant melanoma, Bowen's disease, basal cell carcinoma, benign intraductal papilloma, nevoid hyperkeratosis of the nipple and areola (NHNA), squamous metaplasia of lactiferous ducts (SMOLD)/ Zuska's disease and pagetoid dyskeratosis. The prognosis for people with<unk>  is primarily determined by the underlying tumor. Unfavorable prognosis is seen in cases with palpable breast tumor, enlarged lymph nodesand in patients younger than 60 years of age. Prognosis is worse in males than in females. Mean survival was found to be 80.0 months for males and 108.2 months for females. Five-year survival rate has been reported to be 20-30% in males, compared to 30-40% in females. This has been hypothesized to be due to the small size of mammary gland in males. A full-thickness biopsy of the nipple and areola is important for establishing the diagnosis of<unk> . Findings on biopsy diagnostic of<unk>  include presence of Paget cells, which are arranged in solid groups.Ultrasonography of the breast may be performed to detect the breast cancer associated with<unk> . It must be considered as a part of the initial evaluation in patients with negative mammography. MRI may be performed to detect the breast cancer associated with<unk> . In the setting of clinically and mammographically occult Paget's disease MRI can be very useful, mainly since mammography and ultrasonography have limitations in the evaluation of patients with Paget's disease. MRI also has a role in the preoperative evaluation of patients and might be healpful in facilitating treatment decisions. Mammogram may be performed to detect the breast cancer associated with<unk> , but is not always a reliable procedure for detecting it as it has limited reliability in the detection of underlying DCIS in people with this disease. The use of touch/scrape smears for cytological diagnosis is recommended to prevent delay in diagnosis. Mainly in patients who are reluctant to undergo other diagnostic procedures such as wedge biopsy, shave biopsy, surgical excision. A negative result does not exclude the diagnosis of<unk> . In vivo reflectance confocal microscopy allows visualization of the upper layers of the skin at a cellular resolution, may also assist in the early diagnosis of Paget's disease in reluctant patients.Chemotherapy and radiotherapy are indicated for<unk>  as adjuvant therapy or palliative treatment in patients with underlying ductal carcinoma or invasive breast cancer. Mastectomy is the mainstay of treatment for<unk> . Patients who do not have a palpable lump are treated with removal of the nipple and areola, followed by whole-breast radiation therapy, whereas patients with associated ductal carcinoma in situ or invasive breast cancer are treated with complete resection of the underlying disease with excision of the nipple-areola complex and radiation therapy of the remaining breast tissue. When lymph nodes are involved, more extensive axillary lymph node surgery may be needed. Adjunctive radiation may be required.
Paget's disease of the breast
Papillary carcinoma of the breast
Duct ectasia of breast
Mammary ductal carcinoma
Inflammatory breast cancer
Mammary adenosis
Breast abscess
Extramammary Paget's disease