Papular XXXX with "skyline" basal cell layer (PENS).

@entity1 @entity2557 @entity2611 @entity9397 @entity4357
BACKGROUND: Several types of epidermal keratinocytic nevus are recognized. OBJECTIVE: We sought to describe a previously unreported keratinocytic nevus with distinctive clinical and histopathologic features in 5 @entity1 . METHODS: We performed a clinical and photographic review, and obtained skin biopsy samples for histopathologic examination from each @entity1 . Genetic analysis to screen for @entity2557 and @entity4357 3-kinase, catalytic, alpha hotspot mutations was performed on lesional skin from two @entity1 . RESULTS: Five @entity1 (2 male, 3 female) had from 1 to 11 lesions present since birth. These consisted of 1- to 7-mm hyperkeratotic papules with a rough, flat surface and a round, commalike, rectangular, or polygonal shape. Histopathologic examination showed acanthosis with @entity2611 , and strikingly arranged basal cells with palisaded nuclei. Genetic testing on paraffin-embedded specimens from two @entity1 ruled out hotspot mutations in the @entity2557 and @entity4357 3-kinase, catalytic, alpha genes. LIMITATIONS: A small number of @entity1 are presented. CONCLUSION: We propose the name "papular @entity9397 with 'skyline' basal cell layer" (PENS) for this newly recognized condition.
