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European Journal of Dermatology

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Phacomatosis pigmentokeratotica: another epidermal nevus syndrome and a distinctive type of twin spotting Volume 10, issue 3, April - May 2000

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Authors
Department of Dermatology, Hospital del Niño Jesús, Pasaje Bertres 224, San Miguel de Tucumán, 4000 Tucumán, Argentina.

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.