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  • Title: Phacomatosis pigmentokeratotica: another epidermal nevus syndrome and a distinctive type of twin spotting.
    Author: Boente MC, Pizzi de Parra N, Larralde de Luna M, Bonet HB, Santos Muñoz A, Parra V, Gramajo P, Moreno S, Asial RA.
    Journal: Eur J Dermatol; 2000; 10(3):190-4. PubMed ID: 10725816.
    Abstract:
    The name epidermal nevus syndrome could be applied to a group of clinically and histopathologically different entities as has been pointed out by Happle. Phacomatosis pigmentokeratotica is a further type of epidermal nevus syndrome distinguished by the presence of a sebaceous nevus and a contralateral speckled lentiginous nevus of the papular type, associated with skeletal or neurological abnormalities. Three new cases of this recently delineated syndrome are presented. A common origin may account for the temporal and spatial relationship between the epidermal and the speckled lentiginous nevus. The concept of melanocytic-epidermal twin spotting similar to the interpretation of vascular twin spotting could explain the pathogenesis of this entity.
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