A Case of Paget's Disease of the Breast without a Nipple Lesion

Paget's disease of the breast is generally associated with breast cancer involving the epithelium of the nipple and frequently extends into the areola or surrounding epidermis. Although it classically presents as a nipple lesion, recent diagnostic frameworks recognize that Paget's disease may rarely occur without overt nipple involvement. We report a rare case of Paget's disease of the breast without a nipple lesion.

The patient was a 73-year-old woman who was referred to the dermatology department at our hospital for investigation of a ring-shaped area of erythema on her right areola that had been treated with topical steroids for a month at a local dermatology clinic with no improvement. A skin biopsy led to a diagnosis of Paget's disease of the breast, and she was referred to our department. Mammography and ultrasonography did not show any intramammary lesions, while MRI suggested possible limited ductal involvement near the nipple. However, the exact extent of the disease could not be reliably delineated preoperatively. A right mastectomy was performed. Postoperative pathology confirmed Paget cells in the erythematous area and ductal carcinoma in situ within the breast, without demonstrable histological continuity between the two lesions.

When a patient presents with a refractory lesion in the areolar region, even in the absence of nipple involvement, the possibility of Paget's disease of the breast should be considered, and a skin biopsy should be performed for appropriate diagnosis.