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Basal cell carcinomas (BCCs) are malignant, locally invasive, slow-growing tumors that arise from basal cells and represent 75% of non-melanoma skin cancers. BCC has many different clinical presentations, but the most common types include nodular, micronodular, superficial, morpheaform, infiltrative, and fibroepithelioma of Pinkus. These neoplasms develop in 80% of cases in sun-exposed areas of the head and neck, and only a minority of cases have been reported arising in the nipple-areola complex. In these locations, Paget’s disease of the breast has to be first ruled out before BCC could be considered. Furthermore, since most BCCs occur in adult age, cases that appear earlier in childhood with increased number and unusual distribution should be checked for genetic disorders such as Basal cell nevus syndrome.
Thus, we reported an original case of superficial BCC of the nipple-areola complex masquerading as Paget’s disease of the breast and revealing a Basal cell nevus syndrome.
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