Abstract:

An African American female in her thirties presented to the Dermatology clinic for initial evaluation of a hyperpigmented area overlying a hypoplastic right breast. The hyperpigmented area first appeared in middle school prior to puberty. Her left, uninvolved breast continued to develop throughout puberty whereas her right breast was halted in development ipsilateral to the side of the hyperpigmented lesion. She had previously undergone extensive work up with fine needle aspiration of the hypoplastic right breast which demonstrated a complex radial sclerosing lesion including radial scar, florid ductal hyperplasia with fibroadenomatoid changes with sclerosing adenosis, and cystic apocrine metaplasia. She recently underwent a breast reduction of the left, uninvolved breast to obtain better symmetry. On physical exam, on her right breast there was a large well demarcated brown pigmented plaque extending towards the right axillae. A four-millimeter punch biopsy was obtained from the center of the hyperpigmented plaque. On H&E there was acanthosis and hyperpigmentation of the epidermis. There was an increase in the number of smooth muscle bundles in the dermis. Overall, these findings were consistent with Beckers Nevus Syndrome, given the histopathological signs of Beckers nevus in association with ipsilateral breast hypoplasia. Beckers nevus is a variant of an epidermal nevus that is androgen dependent. Beckers Nevus Syndrome involves the presence of Beckers Nevus with associated breast hypoplasia or dysplasia of skeletal, muscle, or subcutaneous tissues. Similar to other epidermal nevi, this is thought to arise due to mosaicism and postzygotic mutations involved embryo can lead to paradominant inheritance. Anti-androgen agents have been investigated as a possible therapy, although clinical improvement is limited once the nevus is fully developed.

Financial Disclosure:
No current or relevant financial relationships exist.

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Lindsey West, MD

Biography