Dermatologic Mimickers of Paget’s Disease of the Breast: A Systematic Review

Abstract

Mammary Paget disease (MPD) is a rare form of breast cancer that accounts for just 1% to 4% of all cases and is often associated with underlying malignancies such as ductal carcinoma in situ and invasive ductal carcinoma. Its clinical presentation frequently mimics benign dermatologic conditions or malignant melanoma, leading to diagnostic confusion and significant treatment delays. This review explores the diagnostic challenges and patterns of misdiagnosis in MPD, as well as the consequences of delayed recognition. A comprehensive search of Embase and MEDLINE identified 29 studies reporting on 32 cases of MPD, all of which were initially misdiagnosed—most commonly as melanoma (44.4%) and atopic dermatitis (25.0%). The average diagnostic delay was 2.3 years. Most lesions were unilateral (93.8%) and involved the nipple-areolar complex (87.5%). Imaging modalities demonstrated limited sensitivity, reinforcing the importance of early biopsy for timely diagnosis. Surgical intervention was the predominant treatment approach, employed in 75% of cases, and no recurrences were reported during a mean follow-up of 1.3 years. These findings underscore the urgent need for heightened clinical suspicion, earlier tissue sampling, and the development of standardized diagnostic pathways to reduce misdiagnosis and improve outcomes in patients with MPD.