Pigmented Paget disease of the nipple is a rare disease. We report on a 60-year-old woman in whom this diagnosis was made based on clinical findings, dermoscopy, histology and immunohistology. Circumscribed pigmentation of the nipple includes a wide spectrum of benign and malignant differential diagnostic considerations. Dermoscopy can enhance the clinical diagnosis. Biopsies should always be performed on unclear pigmentation of the nipple in both women and men. On histology, rare melanocytic tumors must be differentiated from pigmented Paget disease and pigmented Bowen disease. Pagetoid intraepithelial cells of a melanocytic tumor are positive for S100, HMB45 or melan A, but not for cytokeratins. Intraepithelial tumor cells of Paget disease are positive for cytokeratin 7. The clear cells in Bowen diseases are usually not positive for cytokeratin 7 and are also negative for all melanocytic markers.
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