Paget's disease of the breast (PDB) is a carcinoma of the breast that involves the nipple and the areola. It is a rare disease and is usually linked to carcinoma in situ, or an invasive carcinoma in the breast, which may be evidenced by a palpable mass on examination of the breast [1]. PDB constitutes only about 1-4% of reported cases of breast cancer in females [2]. About 40% of those associated with invasive cancer are associated with ductal carcinoma in situ [3]. PDB can occur in both men and women, however, it predominantly affects the latter. The disease is often diagnosed in the 5th decade, although cases of younger and older-onset have been reported [1]. PDB often presents as unilateral disease. It can occur in accessory nipples, as well as non-breast tissue after mastectomy [4] [5]. Furthermore, it has been reported to occur in the breast tissue in the axilla [6].

PDB affects the skin and causes lesions that may resemble eczema [7]. For this reason, it may be misdiagnosed as a dermatological condition. The nipple and areola are often erythematous, itchy, flaky, and sometimes there is an erosion of the nipple. A nipple discharge is not uncommon and may range from serosanguineous to purulent. Other reported findings are ulceration, bleeding, thick plaque formation, and pain that is often burning in nature.

• Burning Pain

  pain in the affected nipple. [merckmanuals.com]

• Hematochezia

  In such patients, liver disease is not always evident and patients may have fat-soluble vitamin malabsorption with rickets, corrected by vitamin supplementation, and/or other complications including bleeding diathesis (hematochezia or intracranial bleeding [orpha.net]

• Cutaneous Manifestation

  J Can Res Ther 2010;6:95-6 Introduction Since the initial description in 1874, 2 Cutaneous manifestations of male breast cancer Reginster, M.A., Quatresooz, P., Piérarrarrard-Fraranchimont, C., Piérarrarrard, G.E. [cancerjournal.net]

  Abstract Paget's disease of the breast nipple and areola complex represents a cutaneous manifestation of an underlying breast malignancy. [ncbi.nlm.nih.gov]

  There are several clinical cutaneous manifestations of psoriasis but most commonly the disease presents as chronic, symmetrical, erythematous, scaling papules and plaques. [farethrea.duckdns.org]

  Sánchez, Cutaneous Manifestations of Internal Malignancy and Paraneoplastic Syndromes, Atlas of Dermatology in Internal Medicine, 10.1007/978-1-4614-0688-4_6, (59-76), (2011). D. Geffroy, I. Doutriaux-Dumoulins, C. Labbe-Devilliers, P. Meingan, S. [doi.org]

• Leg Cramp

  Raloxifene can cause side effects including flu-like symptoms, hot flushes and leg cramps. Side effects of tamoxifen can include hot flushes and sweats, changes to your periods and nausea and vomiting. [nhsdirect.wales.nhs.uk]

• Suggestibility

  The other theory suggests that skin cells of the nipple spontaneously become Paget cells. [web.archive.org]

  Your doctor will do a physical exam and should suggest having a mammogram of both breasts done immediately. [truthorfiction.com]

  The areola lesion was highly suggestive of a dermatologic malignancy such as malignant melanoma. As such, a biopsy was performed with initial findings suggestive of cutaneous melanoma. [ncbi.nlm.nih.gov]

• Anger

  Prolonged stress may lead to frustration, anger, hopelessness, and -- at times -- depression. The person with the illness is not the only one affected. Family members are also influenced by the health changes of a loved one. [web.archive.org]

  You may have many different emotions, including anger, resentment, guilt, anxiety and fear. These are all normal reactions and are part of the process many people go through in trying to come to terms with their condition. [macmillan.org.uk]

• Nipple Discharge

  Paget Disease of the Breast Nipple Discharge Breast discharge is the spontaneous flow of fluid from the nipple at any time other than during nursing. [medicinenet.com]

  The symptoms include: Itching, tingling, redness, and/or a burning sensation in the affected nipple Sensitivity and pain Flaking of scales and skin thickening of the affected nipple Flattening or turning inwards (inversion) of the nipple Discharge (yellowish [news-medical.net]

  The most common presenting symptoms were nipple discharge and eczematous changes of the nipple/areola complex. Ninety-seven patients (93.2%) had an underlying invasive or noninvasive cancer associated with Paget's disease. [ncbi.nlm.nih.gov]

  If you have nipple discharge, a sample of the discharge might also be collected for microscopic examination. If cancer cells are detected in the samples collected, you may be referred to a breast surgeon to discuss treatment options. [web.archive.org]

• Ulcer of Nipple

  […] the nipple might flatten out or turn inwards (invert) there may be a discharge or bleeding from the nipple the area might feel itchy or sore there may be a lump in the same breast. [breast-cancer.canceraustralia.gov.au]

Paget's disease of the breast has four stages, stage zero describing malignancy that only involves the epidermis, and stage four being PDB that is associated with invasive ductal carcinoma. There are several modalities that are used in the diagnosis of PDB. Clinical breast examination is of particular importance as up to 50% of those with PDB will have a palpable breast lump. Subsequent diagnostic techniques involve both invasive and noninvasive strategies.

Breast tissue from the affected area should be analyzed histologically, thus necessitating a biopsy. The choice of what type of biopsy to carry out ranges between wedge, punch, shave and surface biopsy. Immunochemistry is also carried out on biopsy samples. Together, the above laboratory techniques (immunohistochemistry) form the definitive diagnostic measure for PDB [8].

Imaging studies include mammography, ultrasound and magnetic resonance imaging (MRI) [1]. These are useful in detecting nonpalpable breast masses [9]. On mammography, PDB may cause distortion of the breast architecture, calcifications, edema, and nipple changes. Negative findings on mammography do not rule out the possibility of PDB, as about 70% of cases with histological evidence of the disease also have positive findings on mammography. Therefore there is a proportion of cases of PDB without mammographic features of the disease. MRI is useful in detecting PDB that otherwise goes undetected by the former [10].

1. Caliskan M, Gatti G, Sosnovskikh I, et al. Paget's disease of the breast: the experience of the European Institute of Oncology and review of the literature. Breast Cancer Res Treat. 2008;112(3):513–521.
2. Subramanian A, Birch H, McAvinchey R, Stacey-Clear A. Pagets disease of uncertain origin: case report. Int Semin Surg Oncol. 2007;4:12.
3. Piekarski J, Kubiak R, Jeziorski A. Clinically silent Paget disease of male nipple. J Exp Clin Cancer Res. 2003;22(3):495-496.
4. Giovannini M, D’Atri C, Piubello Q, Molino A. Mammary Paget's disease occurring after mastectomy. World J Surg Oncol. 2006;4:51.
5. Martin VG, Pellettiere EV, Gress D, Miller AW. Paget's disease in an adolescent arising in a supernumerary nipple. J Cutan Pathol. 1994;21(3):283-286.
6. El Khoury M, Lalonde L, David J, Issa-Chergui B, Peloquin L, Trop I. Paget's disease of the axilla arising from an underlying accessory mammary tissue. Clin Radiol. 2011;66(6):575-577.
7. Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol. 2007;21(5):581–590.
8. Kurokawa I, Furukawa S, Kishimoto M, Kakuno A, Tsubura A. Keratin Expression in Mammary Paget's Disease in situ with Intraductal Invasion. Case Rep Oncol. 2012;5(2):400-403.
9. Echevarria JJ, Lopez-Ruiz JA, Martin D, Imaz I, Martin M. Usefulness of MRI in detecting occult breast cancer associated with Paget's disease of the nipple-areolar complex. Br J Radiol. 2004;77(924):1036-1039.
10. Morrogh M, Morris EA, Liberman L, Van Zee K, Cody HS 3rd, King TA. MRI identifies otherwise occult disease in select patients with Paget disease of the nipple. J Am Coll Surg. 2008;206(2):316-321.