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2014, Número 2

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Cir Plast 2014; 24 (2)


Tratamiento quirúrgico de pseudosindactilia en pacientes con epidermólisis ampollosa distrófica recesiva

Montemayor-Sánchez D, Salas-Alanís JC

Idioma: Español
Referencias bibliográficas: 40
Paginas: 106-112
Archivo PDF: 372.40 Kb.


RESUMEN

La pseudosindactilia de las manos y pies es una complicación inevitable de los pacientes con epidermólisis ampollosa distrófica recesiva. Esta enfermedad hereditaria es el resultado de mutaciones en la colágena tipo VII que forma las fibrillas de anclaje de la membrana basal epidérmica que da como resultado la formación de ampollas al menor traumatismo. La enfermedad progresiva de las manos se caracteriza por la formación de cicatrices deformantes que culmina afectando toda la extremidad dentro de una estructura en forma de capullo. La recurrencia de las lesiones cutáneas y la subsecuente formación de pseudosindactilia son inevitables; el manejo quirúrgico está enfocado en mantener la mano funcional disminuyendo la progresión de la pseudosindactilia en estos pacientes. Este artículo es una revisión de la fisiopatología y manejo quirúrgico de la pseudosindactilia en pacientes con epidermólisis ampollosa distrófica recesiva, con el propósito de mejorar el manejo de esta patología.


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