Paget’s Disease of the Breast (PDB) – A Review


  • Sajad Ahmad Salati Department of Surgery, Unaizah College of Medicine & Medical Sciences, Qassim University, Saudi Arabia
  • Lamees Alsulaim Department of Surgery, Unaizah College of Medicine & Medical Sciences, Qassim University, Saudi Arabia
  • Ajaz A Rather Department Surgery, SKIMS Medical College, Bemina, Srinagar, Kashmir, India



Paget disease, ductal carcinoma in situ, invasive breast cancer, eczema, nipple-areola complex


Paget’s disease of breast is a cutaneous malignancy of the breast involving the nipple-areolar complex that is often associated with underlying neoplastic lesions of breast parenchyma. This condition is often mistaken for a wide range of dermatological conditions, leading to delay in diagnosis. This review article revisits the etiology, clinical presentation, differential diagnosis, diagnostic work-up, natural history, management and prognosis of Paget’s disease of breast.


Valdes EK, Feldman SM. Paget's disease of the breast. Breast J 2006; 12(1): 83. DOI:

Burke MJ, Middleton TO. Mammary Paget disease. J Am Osteopath Assoc 2013; 113(9): 712. DOI:

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): 283-6. DOI:

Yasir M, Khan M, Lotfollahzadeh S. Mammary Paget Disease. [Updated 2022 Jun 3]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https: //

Xu L, Yin S, Wang S, Feng J, Liu L, Liu G, Wang J, Zhan S, Zhao Z, Gao P. Prevalence of mammary Paget's disease in urban China in 2016. Sci Rep 2021; 11(1): 2572. DOI:

Markarian S, Holmes DR. Mammary Paget's Disease: An Update. Cancers (Basel) 2022; 14(10): 2422. DOI:

Di Tommaso L, Franchi G, Destro A, Broglia F, Minuti F, Rahal D, Roncalli M. Toker cells of the breast. Morphological and immunohistochemical characterization of 40 cases. Hum Pathol 2008; 39(9): 1295-300. DOI:

Lopes Filho LL, Lopes IM, Lopes LR, Enokihara MM, Michalany AO, Matsunaga N. Mammary and extramammary Paget's disease. An Bras Dermatol 2015; 90(2): 225-31. DOI:

Dubar S, Boukrid M, Bouquet de Joliniere J, Guillou L, Vo QD, Major A, Ali NB, Khomsi F, Feki A. Paget's Breast Disease: A Case Report and Review of the Literature. Front Surg 2017; 4: 51. DOI:

Szpor J, Polak K, Dyduch G, Okon K, Hodorowicz-Zaniewska D, Brzuzkiewicz K, Streb J. Pigmented Paget's disease of the nipple. Pol J Pathol 2015; 66(1): 93-7. DOI:

Tan AR. Cutaneous manifestations of breast cancer. Semin Oncol 2016; 43(3): 331-4. DOI:

Sakorafas GH, Blanchard K, Sarr MG, Farley DR. Paget's disease of the breast. Cancer Treat Rev 2001; 27(1): 9-18. DOI:

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): 575-7. DOI:

Oiso N, Kawara S, Inui H, Kawada A. Pigmented spots as a sign of mammary Paget's disease. Clin Exp Dermatol 2009; 34(1): 36-8. DOI:

Zou Y, Zhu X, Yang L, Gu J, Xia R. Pigmented mammary Paget disease mimicking melanoma on reflectance confocal microscopy. Skin Res Technol 2020; 26(6): 954-955. DOI:

del Boz J, Sanz A, Martín T, Martínez S, Sánchez V, Samaniego E, Ojeda A, Vera A, Crespo V. Pigmented mammary Paget's disease. J Eur Acad Dermatol Venereol 2007; 21(6): 844-6. DOI:

Chen YC, Chen YJ, Yang CS, Shen JL. A hyperpigmented plaque on the right nipple: a quiz. Pigmented mammary Paget's disease. Acta Derm Venereol 2011; 91(5): 607-8. DOI:

Dalberg K, Hellborg H, Wärnberg F. Paget's disease of the nipple in a population-based cohort. Breast Cancer Res Treat 2008; 111(2): 313-9. DOI:

Kawase K, Dimaio DJ, Tucker SL, Buchholz TA, Ross MI, Feig BW, Kuerer HM, Meric-Bernstam F, Babiera G, Ames FC, Singletary SE, Hunt KK. Paget's disease of the breast: there is a role for breast-conserving therapy. Ann Surg Oncol 2005; 12(5): 391-7. DOI:

Challa VR, Deshmane V. Challenges in Diagnosis and Management of Paget's Disease of the Breast-a Retrospective Study. Indian J Surg 2015; 77(Suppl 3): 1083-7. DOI:

Lim HS, Jeong SJ, Lee JS, Park MH, Kim JW, Shin SS, Park JG, Kang HK. Paget disease of the breast: mammographic, US, and MR imaging findings with pathologic correlation. Radiographics 2011; 31(7): 1973-87. DOI:

Kothari AS, Beechey-Newman N, Hamed H, Fentiman IS, D'Arrigo C, Hanby AM, Ryder K. Paget disease of the nipple: a multifocal manifestation of higher-risk disease. Cancer 2002; 95(1): 1-7. DOI:

Sripathi S, Ayachit A, Kadavigere R, Kumar S, Eleti A, Sraj A. Spectrum of Imaging Findings in Paget's Disease of the Breast-A Pictorial Review. Insights Imaging 2015; 6(4): 419-29. DOI:

Amano G, Yajima M, Moroboshi Y, Kuriya Y, Ohuchi N. MRI accurately depicts underlying DCIS in a patient with Paget's disease of the breast without palpable mass and mammography findings. Jpn J Clin Oncol 2005; 35(3): 149-53. DOI:

Bhadani PP, Sharma MC, Sah SP, Agarwal A. Paget's disease of the nipple diagnosed on cytology: a case report. Indian J Pathol Microbiol 2004; 47(2): 246-8.

Herbst E, Tismenetsky M, McIntosh VM. Unusual case of Paget disease of the nipple diagnosed by nipple scraping cytology. Dermatol Online J 2020; 26(3): 13030/qt0b64c7mh. DOI:

Karakas C. Paget's disease of the breast. J Carcinog 2011; 10: 31. DOI:

Yao DX, Hoda SA, Chiu A, Ying L, Rosen PP. Intraepidermal cytokeratin 7 immunoreactive cells in the non-neoplastic nipple may represent interepithelial extension of lactiferous duct cells. Histopathology 2002; 40(3): 230-6. DOI:

Kanitakis J. Solid cutaneous tumours. In: Kanitakis J, Vassileva S, Woodley D, editors. Diagnostic Immunohistochemistry of the Skin. An Illustrated Text. London: Chapman and Hall Medical; 1998; pp. 279-99.

Rosen PP. Rosen's breast pathology. 2nd ed. Philadelphia: Lippincott-Raven; Paget's disease of the nipple; 2001; pp. 565-80.

Fu W, Lobocki CA, Silberberg BK, Chelladurai M, Young SC. Molecular markers in Paget disease of the breast. J Surg Oncol 2001; 77(3): 171-8. DOI:

Sek P, Zawrocki A, Biernat W, Piekarski JH. HER2 molecular subtype is a dominant subtype of mammary Paget's cells. An immunohistochemical study. Histopathology 2010; 57(4): 564-71. DOI:

Giger O, Caduff R, O'Meara A, Diener PA, Knuth A, Jäger D, Moch H, Varga Z. Frequent expression of the breast differentiation antigen NY-BR-1 in mammary and extramammary Paget's disease. Pathol Int 2010; 60(11): 726-34. DOI:

D'Erme AM, Lozzo R, Viacava P, De Luca F, Janowska A, Dini V, Romanelli M, Fidanzi C, Bagnoni G. Pigmentary mammary Paget disease: Clinical, dermoscopical and histological challenge. Dermatol Reports 2021; 13(3): 9235. DOI:

Choridah L, Sari WK, Dwianingsih EK, Widodo I, Suwardjo, Anwar SL. Advanced lesions of synchronous bilateral mammary Paget's disease: a case report. J Med Case Rep 2020; 14(1): 119. DOI:

Franceschini G, Masetti R, D'Ugo D, Palumbo F, D'Alba P, Mulè A, Costantini M, Belli P, Picciocchi A. Synchronous bilateral Paget's disease of the nipple associated with bilateral breast carcinoma. Breast J 2005; 11(5): 355-6. DOI:

Ucar AE, Korukluoglu B, Ergul E, Aydin R, Kusdemir A. Bilateral Paget disease of the male nipple: first report. Breast 2008; 17(3): 317-8. DOI:

Fox S, Speirs V, Shaaban AM. Male breast cancer: an update. Virchows Arch 2022; 480(1): 85-93. DOI:

El Harroudi T, Tijami F, El Otmany A, Jalil A. Paget disease of the male nipple. J Cancer Res Ther 2010; 6(1): 95-6. DOI:

Akita M, Kusunoki N, Nakajima T, Takase S, Maekawa Y, Kajimoto K, Ohno M. Paget's disease of the male breast: a case report. Surg Case Rep 2015; 1(1): 103. DOI:

Uthamalingam M, Periyasamy K. Paget's Disease of Nipple in Male Breast with Cancer. J Clin Diagn Res 2016; 10(2): PD14-6. DOI:

Vergine M, Musella A, Gulotta E, Frusone F, De Luca A, Maceli F, Libia A, Benedetti Panici P, Monti M. Paget's disease of the male breast: case report and a point of view from actual literature. G Chir 2018; 39(2): 114-117.

Pourmoussa A J, Mautner S K, Nasseri-Nik N, Sachar KL. Invasive Paget’s Disease of the Breast: Rash or Recurrence? Cureus 2021; 13(11): e19216. DOI:

Plastaras JP, Harris EE, Solin LJ. Paget's disease of the nipple as local recurrence after breast-conservation treatment for early-stage breast cancer. Clin Breast Cancer 2005, 6: 349-53. DOI:

Marshall JK, Griffith KA, Haffty BG, Solin LJ, Vicini FA, McCormick B, Wazer DE, Recht A, Pierce LJ. Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results. Cancer 2003; 97(9): 2142-9. DOI:

Fourquet A, Campana F, Vielh P, Schlienger P, Jullien D, Vilcoq JR. Paget's disease of the nipple without detectable breast tumor: conservative management with radiation therapy. Int J Radiat Oncol Biol Phys 1987; 13(10): 1463-5. DOI:

Stockdale AD, Brierley JD, White WF, Folkes A, Rostom AY. Radiotherapy for Paget's disease of the nipple: a conservative alternative. Lancet 1989; 2(8664): 664-6. DOI:

Bulens P, Vanuytsel L, Rijnders A, van der Schueren E. Breast conserving treatment of Paget's disease. Radiother Oncol 1990; 17(4): 305-9. DOI:

Nardelli AA, Stafinski T, Menon D. Effectiveness of photodynamic therapy for mammary and extra-mammary Paget's disease: a state of the science review. BMC Dermatol 2011; 11: 13. DOI:

Kollmorgen DR, Varanasi JS, Edge SB, Carson WE 3rd. Paget's disease of the breast: a 33-year experience. J Am Coll Surg 1998; 187(2): 171-7. DOI:

Zhou H, Lu K, Zheng L, Guo L, Gao Y, Miao X, Chen Z, Wang X. Prognostic significance of mammary Paget's disease in Chinese women: a 10-year, population-based, matched cohort study. Onco Targets Ther 2018; 11: 8319-8326. DOI:




How to Cite

Salati, S. A., Alsulaim, L., & Rather, A. A. (2022). Paget’s Disease of the Breast (PDB) – A Review. Journal of Analytical Oncology, 11, 45–53.