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Papular Epidermal Nevus with “Skyline” Basal Cell Layer (PENS) with Extracutaneous Findings


Address correspondence to Antonio Torrelo, M.D., Department of Dermatology, Hospital del Niño Jesús, Menéndez Pelayo 65, 28009-Madrid, Spain, or e-mail:


Abstract:  Papular epidermal nevus with “skyline” basal cell layer (PENS), a variant of epidermal nevus, was recently described in otherwise normal children. We describe herein a patient with multiple, typical PENS lesions associated with peculiar facies, bilateral Achilles tendon shortening, and mild psychomotor delay. The association of PENS with extracutaneous manifestations suggests the possibility of a new type of epidermal nevus syndrome, for which we propose the term PENS syndrome.