Paget disease of the nipple (mammary Paget disease)

Synonyms
Mammärer Morbus Paget, Morbus Paget der Brustwarze, Paget-Karzinom der Brust, Mammary Paget disease, Paget disease of the breast, Paget's disease of the nipple
Specialty
Dermatology · Skin tumours
Images
Clinical 1 · Histology 1
In the app
1 flashcards
Last updated
10/2026 · Dr. Pascal Bafteh
Contents
  1. Images (2)
  2. Definition
  3. Occurrence & epidemiology
  4. Aetiopathogenesis
  5. Clinical features
  6. Diagnosis
  7. Differential diagnoses
  8. Keep learning in the app
  9. References (selection)
  10. Cross-references

Images (2)

Paget disease of the nipple (mammary Paget disease) – AI illustration (not a patient photo): unilateral, well-defined, erosive-scaling and oozing erythematous plaque on the nipple and areolaAI illustration
unilateral, well-defined, erosive-scaling and oozing erythematous plaque on the nipple and areolaAI-generated illustration for teaching purposes – not a real patient photo.
Paget disease of the nipple (mammary Paget disease) – histology (H&E): large pale Paget cells with abundant cytoplasm, singly and in nests within the nipple epidermisHistology
histology (H&E): large pale Paget cells with abundant cytoplasm, singly and in nests within the nipple epidermisSarahkayb, „Breast PagetsDisease PA", CC BY-SA 4.0, via Wikimedia Commons · CC BY-SA 4.0 · Source
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Definition

Mammary Paget disease is an uncommon form of breast cancer in which tumour cells (Paget cells) infiltrate the epidermis of the nipple and areola. It is nearly always associated with an underlying intraductal carcinoma, in situ or invasive. It is distinct from extramammary Paget disease of the apocrine gland-bearing regions (anogenital skin, axillae).

Occurrence & epidemiology

It accounts for 1–3% of all breast cancers. It mainly affects women in their 50s and 60s, with a wide age range; men are rarely affected.

Aetiopathogenesis

According to the epidermotropic theory, Paget cells arise in an intraductal carcinoma and migrate along the lactiferous ducts into the nipple epidermis. Another theory regards them as malignantly transformed keratinocytes of the nipple skin.

Clinical features

  • Usually a unilateral eczema- or psoriasis-like change of the nipple that slowly extends over the areola; a well-defined but irregularly shaped red patch, scaly or crusted, up to 15 cm across.
  • Initially asymptomatic, later itchy, burning or painful; the nipple may become thickened or retracted; erosion, bleeding and discharge can occur.
  • About 50–60% of patients have a palpable breast lump at presentation.
  • A rare pigmented variant is clinically difficult to distinguish from melanoma.

Diagnosis

  • Suspected with a longstanding, slowly progressive, unilateral change of the nipple.
  • Skin biopsy: typical foamy Paget cells in the epidermis; in the pigmented variant, immunohistochemistry (e.g. CK7, S100, Melan-A, HMB-45) to distinguish them from melanoma cells. A wedge biopsy of the nipple also samples deeper breast tissue.
  • Breast imaging (ultrasound, mammography, MRI) to look for the underlying carcinoma and determine its extent.

Differential diagnoses

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References (selection)

  1. DermNet: Mammary Paget disease
  2. MSD Manual Professional: Breast Cancer (Abschnitt Paget disease of the nipple)

Cross-references

Mentioned in

More skin tumours

Note: This page is intended for medical education and does not replace diagnosis or treatment decisions in individual cases.