Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences

View all Images (28)

Paget disease of breast
Other Resources UpToDate PubMed

Paget disease of breast

Contributors: Vivian Wong MD, PhD, Whitney A. High MD, JD, MEng, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Mammary Paget disease (MPD) is a malignant intraepithelial condition of the nipple and periareolar skin that occurs nearly exclusively in women, usually when breast carcinoma is present. Postmenopausal women after the sixth decade of life are most commonly affected, although adolescent and elderly patients may develop MPD as well. MPD in men is extremely rare. Clinically, MPD presents as a unilateral eczematous or crusted lesion on the nipple or areola that develops slowly over months to years. Bilateral cases are rare but have been reported.

It is widely accepted that MPD is nearly always associated with underlying breast carcinoma. Malignant cells from the underlying ductal adenocarcinoma invade the nipple unit and overlying periareolar skin. The malignant cells spread through the normal epidermis in "pagetoid fashion," admixed among normal keratinocytes.

Only 1%-4% of breast carcinoma presents with MPD, but nearly 100% of MPD is associated with an underlying breast carcinoma (ductal carcinoma in situ [10%] or infiltrating carcinoma [90%]).

The malignant cells of MPD express the same apomucins and possess the chromosomal aberrations of the underlying breast cancer. The condition is detected on biopsy using the same immunohistochemical stains employed in breast carcinoma, such as cytokeratin 7 (CK7).

Presence of a palpable mass in the breast, lymphadenopathy, younger age (less than 60 years), and aggressive histology may be associated with poor prognosis. The presence of a palpable mass in the breast may represent invasive carcinoma rather than in-situ stage.

Treatment requires referral to a breast oncologist and oncologic surgeon to detect and manage the underlying breast carcinoma.

Related topics: cutaneous presentations of breast cancer, female breast cancer, male breast cancer

Codes

ICD10CM:
C50.019 – Malignant neoplasm of nipple and areola, unspecified female breast

SNOMEDCT:
403946000 – Paget's disease of nipple

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

To perform a comparison, select diagnoses from the classic differential

Subscription Required

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:07/15/2024
Last Updated:08/14/2024
Copyright © 2024 VisualDx®. All rights reserved.
Paget disease of breast
A medical illustration showing key findings of Paget disease of breast : Breast, Breast pain, Burning skin sensation, Erythema, Scaly plaque, Pruritus, Nipple/areola
Clinical image of Paget disease of breast - imageId=5381923. Click to open in gallery.  caption: 'A macerated, eroded, and crusted plaque over the nipple and areola.'
A macerated, eroded, and crusted plaque over the nipple and areola.
Copyright © 2024 VisualDx®. All rights reserved.