Phacomatosis pigmentokeratotica: another epidermal nevus syndrome and a distinctive type of twin spotting

The name epidermal news syndrome could be applied to a group of clinically and histopathologically different entities as has been pointed out by Happl e. Phacomatosis pigmentokeratotica is a further type of epidermal nevus syn drome distinguished by the presence of a sebaceous nevus and a contralatera l 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 betwe en the epidermal and the speckled lentiginous nevus. The concept of melanoc ytic-epidermal twin spotting similar to the interpretation of vascular twin spotting could explain the pathogenesis of this entity.