title: Phacomatosis pigmentokeratotica: another epidermal nevus syndrome and a distinctive type of twin spotting.
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.
authors.affiliation: Department of Dermatology, Hospital del Niño Jesús, Pasaje Bertres 224, San Miguel de Tucumán, 4000 Tucumán, Argentina.
authors.forename: M C
authors.initials: MC
authors.lastname: Boente
authors.forename: N
authors.initials: N
authors.lastname: Pizzi de Parra
authors.forename: M
authors.initials: M
authors.lastname: Larralde de Luna
authors.forename: H B
authors.initials: HB
authors.lastname: Bonet
authors.forename: A
authors.initials: A
authors.lastname: Santos Muñoz
authors.forename: V
authors.initials: V
authors.lastname: Parra
authors.forename: P
authors.initials: P
authors.lastname: Gramajo
authors.forename: S
authors.initials: S
authors.lastname: Moreno
authors.forename: R A
authors.initials: RA
authors.lastname: Asial
mesh_terms: D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D020752:Neurocutaneous Syndromes / Q000473:pathology*; D009506:Nevus / Q000473:pathology*; D013577:Syndrome