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Vol. 110. Num. 1.January - February 2019
Pages e1-e6Pages 1-86
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Vol. 110. Num. 1.January - February 2019
Pages e1-e6Pages 1-86
Brief Comunication
DOI: 10.1016/j.adengl.2018.11.004
Recessive Dystrophic Epidermolysis Bullosa and Pregnancy
Epidermólisis bullosa distrófica recesiva y embarazo
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F. Boriaa,
Corresponding author
F.Boria.Alegre@gmail.com

Corresponding author.
, R. Masedab, M. Martín-Cameána, M. De la Callea, R. de Lucasb
a Unidad de Medicina Materno-Fetal, Servicio de Obstetricia, Hospital Universitario La Paz, Madrid, Spain
b Servicio de Dermatología, Hospital Universitario La Paz, Madrid, Spain
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Abstract

Dystrophic epidermolysis bullosa is a rare inherited disease caused by mutations in the COL7A1 gene. Its recessive variant (recessive dystrophic epidermolysis bullosa) is characterized by the absence or considerably reduced expression of type VII collagen, which leads to marked fragility of the skin and mucous membranes and subsequent blister formation, whether spontaneously or following minimal injury. There have been very few reports of this disease in pregnant women.

We present 2 cases of pregnant women with recessive dystrophic epidermolysis bullosa managed in our High-Risk Pregnancy Unit at Hospital Universitario La Paz, Madrid, Spain. Both patients underwent full-term cesarean delivery, with no further complications for mother or child.

Although recessive dystrophic epidermolysis bullosa increases the risk of maternal complications, a patient is not advised against pregnancy. With adequate monitoring, these patients can fulfil their desire to become mothers.

Keywords:
Epidermolysis bullosa
Pregnancy
Female
Pregnancy complications
Dystrophic epidermolysis bullosa
Resumen

La epidermólisis bullosa distrófica es una enfermedad hereditaria rara debida a mutaciones del gen COL7A1. Su variante recesiva (EBDR) se caracteriza por una marcada disminución o ausencia completa de colágeno tipo VII (C7), que da lugar a una marcada fragilidad de la piel y las mucosas, desencadenando la formación de ampollas de forma espontánea o en respuesta a mínimos traumatismos. Son muy pocos los casos descritos en la literatura de esta enfermedad en embarazadas.

Exponemos 2 casos de gestantes, ambas afectadas de EBDR, y su manejo en nuestra Unidad de Obstetricia de Alto Riesgo del Hospital Universitario La Paz. En ambos casos se realizó una cesárea a término, finalizando la gestación sin complicaciones mayores para la madre o el feto.

A pesar de relacionarse con un mayor número de complicaciones maternas, la EBDR no representa una contraindicación para la gestación, y con un control adecuado, estas pacientes pueden ver su deseo genésico cumplido.

Palabras clave:
Epidermólisis bullosa
Embarazo
Mujer
Complicaciones del embarazo
Epidermólisis bullosa distrófica

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