Mammary Paget's disease

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 2E65.5

1/5

Morbus Paget, cancer eczema, Paget`s disease, Paget`s disease of the nipple.

Unilateral, intraepidermal, usually intraductal adenocarcinoma of the nipple.

  • Very rare in men
  • Peak in age: after 40 years
.

Cause is unclear.

Unsharp, erythematous, unilateral, indolent, weeping, possibly slightly scaly, centrifugally growing, partly eczematous, partly ulcerating papule or plaque in the area of the nipple. Retraction of the nipple may occur. In some cases, subcutaneous nodules are palpable

  • Clinical image
    • Any unilateral eczematous skin lesion in the region of the nipple should be biopsied.
  • Palpation
    • Breast
    • Locoregional lymph nodes
  • Staging, if indicated
  1. Helme S, Harvey K, Agrawal A. Breast-conserving surgery in patients with Paget's disease. Br J Surg 2015;102:1167-74. 
  2. Tang X, Umemura S, Kumaki N, et al. A case report of pigmented mammary Paget's disease mimicking nevus of the nipple. Breast Cancer 2014;21:370-4. 
  3. Lopes Filho LL, Lopes IM, Lopes LR, Enokihara MM, Michalany AO, Matsunaga N. Mammary and extramammary Paget's disease. An Bras Dermatol 2015;90:225-31. 
  4. Vanstapel MJ, Gatter KC, De Wolf-Peeters C, Millard PR, Desmet VJ, Mason DY. Immunohistochemical study of mammary and extra-mammary Paget's disease. Histopathology 1984;8:1013-23. 
  5. Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol 2007;21:581-90.