Abstract
Phacomatosis pigmentokeratotica (PPK) is a rare variant of epidermal nevus syndrome characterized by the combination of a nevus sebaceus and papular speckled lentiginous nevus, usually with associated neurologic, musculoskeletal, and ophthalmologic abnormalities. The nevus sebaceus is arranged along the lines of Blaschko, and the papular speckled lentiginous nevus is typically arranged in a checkerboard pattern. We present a clinically extensive case of cutaneous PPK in a young boy without evidence of extracutaneous abnormalities after more than a decade of follow-up.
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