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Mammary and extramammary Paget's disease
  1. J Lloyd1,
  2. A M Flanagan2
  1. 1Department of Histopathology, Northwick Park and St Mark's NHS Trust, Watford Road, Harrow, Middlesex HA1 3UJ, UK
  2. 2Imperial College School of Medicine (St Mary's Campus), Norfolk Place, London W2 1PG, UK
  1. Dr Lloyd 8spook8{at}


Mammary and extramammary Paget's disease are uncommon intraepithelial adenocarcinomas. Both conditions have similar clinical features, which mimic inflammatory and infective diseases. Histological diagnostic confusion can arise between Paget's disease and other neoplastic conditions affecting the skin, with the most common differential diagnoses being malignant melanoma and atypical squamous disease. The glandular differentiation of both mammary Paget's disease and extramammary Paget's disease is indicated by morphological appearances, the presence of intracellular mucin in many cases, and positive immunohistochemical staining for glandular cytokeratins, epithelial membrane antigen, and carcinoembryonic antigen. This article provides an overview of mammary and extramammary Paget's disease and discusses recent evidence regarding the cell of origin. The concepts of primary and secondary Paget's disease are presented and the differential diagnosis is discussed with reference to immunohistochemical markers that might be of diagnostic value.

  • mammary Paget's disease
  • extramammary Paget's disease

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