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Incontinencia pigmentaria acrómica - All Information

Nombres alternativos

Hipomelanosis de Ito (más comúnmente usado)

Definición:

Es una rara anomalía congénita que causa parches inusuales de piel clara (hipopigmentada) y posibles problemas neurológicos y esqueléticos.

Ver también: hipopigmentación.

Causas:

La causa se desconoce. Es dos veces más común en las niñas que en los niños.

Síntomas:

  • Ojos bizcos (estrabismo)
  • Problemas auditivos
  • Aumento del vello corporal (hirsutismo)
  • Escoliosis
  • Convulsiones
  • Parches con franjas en forma de remolino o con aspecto de mota en brazos, piernas y la parte media del cuerpo
  • Grados variables de retardo

Pruebas y exámenes:

Un examen de las lesiones de la piel con lámpara de Woods puede ayudar a confirmar el diagnóstico. Es probable que el médico recomiende un análisis cromosómico o algún otro tipo de análisis para descubrir cualquier problema médico asociado.

Tratamiento:

No existe tratamiento para la hipopigmentación; sólo se tratan los síntomas. Si se desea, se pueden utilizar cosméticos o ropa para cubrir las manchas hipopigmentadas. Las convulsiones, la escoliosis y otros problemas se tratan en la medida que sea necesario.

Pronóstico:

El pronóstico depende del tipo y severidad de los síntomas que se desarrollen. En la mayoría de los casos, el pigmento de la piel finalmente retorna a la normalidad.

Posibles complicaciones:

  • Molestia y problemas al caminar debido a la escoliosis
  • Angustia emocional relacionada con la apariencia física
  • Retardo mental
  • Convulsiones y posibles lesiones resultantes

Cuándo contactar a un profesional médico:

Consulte con el médico si su hijo presenta un patrón de color de piel inusual.

  • Reviewed last on: 5/13/2011
  • Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network; Linda Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Genodermatoses and Congenital Anomolies. In: James WD, Berger TG, Elston DM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 101th ed. Philadelphia, Pa: Saunders Elsevier; 2011: chap 27.

Moss C. Mosaicism and Linear Lesions. In: Bolognia JL, Jorizzo JL, Rapini RP, eds.: Dermatology. 2nd ed. Philadelphia, Pa: Mosby Elsevier; 2008: chap 61.

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