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