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Fig. 1. --Pequeños tapones hiperqueratósicos distribuidos según las líneas de Blaschko en dorso de manos, dedos, antebrazos y eminencias hipotenares de las palmas. Se puede apreciar un hemangioma sobre el dorso de la mano y muñeca izquierdas.
Fig. 2. --Lesiones en la pierna y talón derechos.
Fig. 3. --Lesiones en la planta del pie izquierdo.
Fig. 4. --Tinción con hematoxilina-eosina (magnificación original, x100). Invaginación epidérmica en cuyo fondo se puede apreciar la pérdida de la capa granulosa. En la parte más profunda se observa un conducto sudoríparo desembocando. En el interior de la invaginación se aprecia un tapón paraqueratósico.
Fig 5. --Tinción con CEA (magnificación original, x100). Obsérvese cómo se tiñe el conducto sudoríparo que se encuentra justo por debajo de la invaginación epidérmica y, sin embargo, no lo hacen las paredes de la misma.
TABLA 1. REVISIÓN DE CASOS CLÍNICOS DE NPODE
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Se considera que el nevo poroqueratósico de los ostios y ductos ecrinos (NPODE) es un hamartoma del conducto sudoríparo. Presentamos el caso de una niña de un mes de edad que presentaba desde el nacimiento una erupción distribuida según las líneas de Blaschko en las extremidades y el tronco, con clara afectación palmoplantar. No se acompañaba de ningún otro defecto congénito y la paciente presentaba un desarrollo normal. La biopsia de una de las lesiones mostró los hallazgos típicos del nevo poroqueratósico de los ostios y ductos ecrinos. La anatomía patológica y las técnicas de inmunohistoquímica en este trastorno parecen revelar que la invaginación epidérmica en donde se halla la lámina paraqueratósica (similar a una lamela cornoide) es atravesada por un acrosiringio aparentemente normal. Dada la distribución de las lesiones y su aparición esporádica, el trastorno parece representar un mosaicismo genético.
Palabras clave:
poroqueratósico, ecrino, ducto, Blaschko, mosaicismo
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is considered to be a hamartoma of the sweat gland duct. We present the case of a one-month-old baby girl who presented from birth with an eruption following Blaschko's lines on the limbs and trunk, with clear palmoplantar involvement. It was not accompanied by any other congenital defect, and the patient's development was normal. The biopsy of one of the lesions showed findings typical of PEODDN. The pathology and immunohistochemistry techniques in this disorder seem to reveal that the epidermal invagination where the Vale column (similar to a cornoid lamella) is found is crossed by an apparently normal acrosyringium. Because of the distribution of the lesions and their sporadic appearance, the disorder seems to represent a genetic mosaicism.
Keywords:
porokeratotic, eccrine, duct, Blaschko, mosaicism
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