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Incontinencia pigmentaria - Overview

Nombres alternativos

Síndrome de Bloch-Sulzberger

Definición:

Es una afección cutánea transmitida de padres a hijos que causa inusual formación de ampollas y cambios en el color de la piel.

Ver también: incontinencia pigmentaria acrómica.

Causas, incidencia y factores de riesgo:

La incontinencia pigmentaria (IP) es causada por una anomalía genética y, en la mayoría de los casos, hay un problema con uno de los genes localizados en el cromosoma X.

Ver también: rasgo ligado al cromosoma X.

La afección se observa con más frecuencia en las mujeres y puede ser mortal cuando ocurre en los hombres.

La mayoría de los bebés que nacen con esta afección desarrollan una decoloración de la piel en las primeras dos semanas. La piel decolorada se presenta cuando una sustancia llamada melanina, que le da el color a la piel, se acumula debajo de ésta.

  • Reviewed last on: 5/13/2011
  • Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network.  Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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