Abstract

Differentiation of pigmented pagetoid lesion of the nipple-areola complex can be very challenging on hematoxylin and eosin-stained sections. Differential diagnoses include melanoma in situ, pigmented mammary Paget’s disease, pigmented epidermotropic breast carcinoma, and pigmented pagetoid Bowen’s disease. Close histologic examination and detailed evaluation of immunohistochemical studies are fundamental tools to achieve a correct diagnosis. We present a case of a 45-year-old woman, who presented an asymptomatic darkly pigmented macule involving the right nipple and areola, suspected of melanoma. Histopathological examination supported by immunohistochemical studies revealed a pigmented mammary Paget’s disease, which is a rarity with only few cases reported. Both clinicians and pathologists need to be aware of this entity and its potential pitfall.

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