Congenital Neurocristic Cutaneous Hamartoma with Poliosis: A Case Report

Abstract

Neurocristic Cutaneous Hamartomas (NCH) are rare, pigmented skin lesions derived from the abnormal migration of neural crest cells. 1 2 We report the case of a 57-year-old female with a congenital localized area of poliosis and underlying pigmented patch on her scalp. Analysis of two punch biopsies yielded features consistent with NCH. Histopathology revealed schwannian-differentiated spindle cells and melanocytic components in the dermis. The spindle cells stained positively with S-100 and the stroma showed a prominent CD34 stain. The melanocytes were positive for melanoma triple stain (HMB-45, Melan A, Tyrosinase) and S-100. As the presentation of NCH and focal poliosis has yet to be described, we will present a literature review of NCH and discuss common features and associations of poliosis.

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