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A variante pigmentada da doença de Paget mamária é rara, com cerca de 12 casos relatados, e pode mimetizar clínica e histologicamente o melanoma. Como na forma clássica, em geral associa-se à neoplasia da mama acometida, com origem principalmente no carcinoma intraductal que se estende à epiderme através de ducto lactóforo. A fisiopatologia da hiperpigmentação permanece desconhecida. Relata-se o caso de paciente de 49 anos que apresentou lesão pigmentada do mamilo, suspeita de melanoma. O exame histológico não foi suficiente para confirmar o diagnóstico, sendo necessária realização de perfil imuno-histoquímico. Apesar de incomum, o diagnóstico de doença de Paget deve ser lembrado como diferencial de melanoma em lesões pigmentadas dessa região, em ambos os sexos. Pigmented mammary Paget's disease is a rare dermatosis, with approximately 12 reported cases in the literature, which can mimic cutaneous melanoma both clinically and histologically. As for classical mammary Paget's disease, the pigmented variant is associated with neoplasia of the breast, originating mainly from intraductal mammary carcinoma that extends to the epidermis of the nipple and areola through a lactiferous duct. Physiopathology of hyperpigmentation remains unknown. We present a 49-year-old female patient who presented with a pigmented lesion of the nipple suspected of melanoma. Histological examination was not sufficient to confirm diagnosis. Immunohistochemical examination confirmed the diagnosis of Paget's disease. Although uncommon, pigmented Paget's disease must be reminded as a differential diagnosis of melanoma in pigmented lesions of the nipple, in both genders

Doença de Paget pigmentada do mamilo simulando melanoma cutâneo: importância da imuno-histoquímica na diferenciação dessas doenças