Paget's disease of the breast in a man with neurofibromatosis

A 68-year-old white man with type 1 neurofibromafosis presented with a 1 -year history of a pruritic non-healing eruption on his left breast. The persistent lesion had been gradually enlarging. This had been unsuccessfully treated with both corticosteroid and antifungal creams. Physical examination of the involved breast revealed a well-demarcated 5 × 3 cm red, slightly Jnfiltrated piaque with adherent fine white scale and erosions with serous exudate (Fig. 1). The areola was completely obscured by the lesion and the nipple could not be identified. Neither a breast mass nor axillary adenopathy was present. A punch biopsy confirmed the diagnosis of Paget's disease. The patient subsequently underwent a left modified radical mastectomy with ipsilateral lymph node dissection. The final pathology revealed intraductal carcinoma, comedo and noncomedo type, with overlying pagetoid cells in the epidermis (Figs 2–4). Special stains included a positive epithelial membrane antigen, focally positive carcinoembryonic antigen, and negative S-100. All 21 lymph nodes submitted from the axillary lymph node dissection were negative for tumor.