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"apellidos" => "de Lucas" ] ] ] ] "resumen" => array:1 [ 0 => array:3 [ "titulo" => "Graphical abstract" "clase" => "graphical" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall"><elsevierMultimedia ident="fig0015"></elsevierMultimedia></p></span>" ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S1578219018304001" "doi" => "10.1016/j.adengl.2018.11.004" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219018304001?idApp=UINPBA000044" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731017306051?idApp=UINPBA000044" "url" => "/00017310/0000011000000001/v1_201901030610/S0001731017306051/v1_201901030610/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S1578219018304074" "issn" => "15782190" "doi" => "10.1016/j.adengl.2018.11.009" "estado" => "S300" "fechaPublicacion" => "2019-01-01" "aid" => "1885" "copyright" => "Elsevier España, S.L.U. and AEDV" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Actas Dermosifiliogr. 2019;110:e1-2" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 2 "HTML" => 2 ] "en" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">E-Case for Diagnosis</span>" "titulo" => "Erythematous Papules on the Dorsum of the Hand" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "e1" "paginaFinal" => "e2" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Pápulas eritematosas en dorso de mano" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 350 "Ancho" => 233 "Tamanyo" => 37586 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin-eosin, magnification ×<span class="elsevierStyleHsp" style=""></span>10. B, Hematoxylin-eosin, magnification ×<span class="elsevierStyleHsp" style=""></span>20.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "C. Maldonado Seral, C. Gómez de Castro, B. Vivanco Allende" "autores" => array:3 [ 0 => array:2 [ "nombre" => "C." "apellidos" => "Maldonado Seral" ] 1 => array:2 [ "nombre" => "C." "apellidos" => "Gómez de Castro" ] 2 => array:2 [ "nombre" => "B." "apellidos" => "Vivanco Allende" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0001731018300073" "doi" => "10.1016/j.ad.2017.05.024" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731018300073?idApp=UINPBA000044" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219018304074?idApp=UINPBA000044" "url" => "/15782190/0000011000000001/v1_201901030631/S1578219018304074/v1_201901030631/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S1578219018304116" "issn" => "15782190" "doi" => "10.1016/j.adengl.2018.11.013" "estado" => "S300" "fechaPublicacion" => "2019-01-01" "aid" => "2026" "copyright" => "Elsevier España, S.L.U. and AEDV" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Actas Dermosifiliogr. 2019;110:43-9" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 2 "HTML" => 2 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Brief Comunication</span>" "titulo" => "Granulomatous Reaction in a Patient With Metastatic Melanoma Treated With Ipilimumab: First Case Reported With Isolated Cutaneous Findings" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "43" "paginaFinal" => "49" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Reacción granulomatosa en paciente con melanoma metastásico tratado con ipilimumab: primer caso descrito presentando clínica cutánea únicamente" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1513 "Ancho" => 1000 "Tamanyo" => 528812 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Pathology findings. Epidermal thinning with irregular acanthosis, discrete spongiosis, and parakeratosis. The papillary dermis is replaced almost entirely by granulomas (A) and granulomatous reaction with prominent macrophages and giant cells (B). (Hematoxylin and eosin, x10.).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "J. Cervantes, A. Rosen, L. Dehesa, G. Dickinson, J. Alonso-Llamazares" "autores" => array:5 [ 0 => array:2 [ "nombre" => "J." "apellidos" => "Cervantes" ] 1 => array:2 [ "nombre" => "A." "apellidos" => "Rosen" ] 2 => array:2 [ "nombre" => "L." "apellidos" => "Dehesa" ] 3 => array:2 [ "nombre" => "G." "apellidos" => "Dickinson" ] 4 => array:2 [ "nombre" => "J." 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Boria, R. Maseda, M. Martín-Cameán, M. De la Calle, R. de Lucas" "autores" => array:5 [ 0 => array:4 [ "nombre" => "F." "apellidos" => "Boria" "email" => array:1 [ 0 => "F.Boria.Alegre@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "R." "apellidos" => "Maseda" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "M." "apellidos" => "Martín-Cameán" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "M." "apellidos" => "De la Calle" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "R." "apellidos" => "de Lucas" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Unidad de Medicina Materno-Fetal, Servicio de Obstetricia, Hospital Universitario La Paz, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario La Paz, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Epidermólisis bullosa distrófica recesiva y embarazo" ] ] "resumenGrafico" => array:2 [ "original" => 1 "multimedia" => array:5 [ "identificador" => "fig0015" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => false "mostrarDisplay" => true "figura" => array:1 [ 0 => array:4 [ "imagen" => "fx1.jpeg" "Alto" => 514 "Ancho" => 1333 "Tamanyo" => 124950 ] ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Epidermolysis bullosa (EB) is a heterogeneous group of hereditary diseases characterized by marked fragility of the skin and mucous membranes and subsequent blister formation, either spontaneously or following minimal injury. EB is a rare disease that can affect people of any ethnic background; men and women are equally affected.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">EB is classified in 4 major types according to the plane of cleavage in the skin: epidermolysis bullosa simplex (EBS), junctional epidermolysis bullosa (JEB), dystrophic epidermolysis bullosa (DEB), and Kindler syndrome (KS).<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">DEB is caused by mutations in the <span class="elsevierStyleItalic">COL7A1</span> gene, which encodes type <span class="elsevierStyleSmallCaps">VII</span> collagen. Type VII collagen is the main component in the anchoring fibrils that link the epithelium to the connective tissue and is therefore essential for maintaining the integrity of the skin and mucous membranes. Severe generalized recessive dystrophic epidermolysis bullosa (RDEB) is the most severe form of EB. It is characterized by a marked decrease in—or complete absence of—type VII collagen, which results in mucosal and cutaneous erosions, chronic wounds, and aggressive squamous cell carcinomas.</p><p id="par0020" class="elsevierStylePara elsevierViewall">There have been very few reports of RDEB in pregnant women due to the significant morbidity associated with these cases. We present 2 cases of pregnant women with RDEB that were managed in our High-Risk Pregnancy Unit at Hospital Universitario La Paz, Madrid, Spain.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Case Descriptions</span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Case 1</span><p id="par0025" class="elsevierStylePara elsevierViewall">The patient was a 40-year-old woman with severe generalized RDEB. Physical examination revealed erosions and blisters on most of the body and pseudosyndactyly of the hands and feet. As an extracutaneous complication, the patient developed esophageal stenosis, which made it difficult to swallow solids. The patient did not have a gastrostomy tube. Her partner was affected by retinosis pigmentaria. Direct sequencing of the <span class="elsevierStyleItalic">COL7A1</span> gene revealed 2 heterozygous mutations in exons 34 and 80. Pregnancy was achieved by in vitro fertilization with the couple's own oocytes and semen because vaginal stenosis prevented sexual relations. Amniocentesis at week 15 of pregnancy ruled out fetal RDEB. As complications of pregnancy, the patient developed hypothyroidism, which was treated with levothyroxine (100<span class="elsevierStyleHsp" style=""></span>μg/d), iron-deficiency anemia, which was treated with intravenous iron (oral administration was impossible due to the patient's difficulty swallowing), and gestational diabetes, which was controlled with diet. The patient also experienced several episodes of secondary infection of the skin lesions with <span class="elsevierStyleItalic">Serratia</span> organisms and <span class="elsevierStyleItalic">Staphylococcus aureus</span>, which responded to antibiotic therapy with amoxicillin–clavulanic acid and cefuroxime (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Follow-up ultrasound at 32 weeks found that the fetus was small for gestational age, with no hemodynamic effects. Cesarean section was performed at week 37<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>4 days due to vaginal stenosis; the infant girl weighed 2200<span class="elsevierStyleHsp" style=""></span>g at birth and was not affected by RDEB. The postsurgical period was without incident and reepithelialization of the surgical wound was achieved after a few weeks. The patient was advised against breastfeeding due to periareolar involvement.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Case 2</span><p id="par0035" class="elsevierStylePara elsevierViewall">The patient was a 25-year-old woman with moderate cutaneous involvement and esophageal dysphagia. Genetic analysis identified 2 heterozygous mutations: the maternal mutation p.R2008C, found on exon 73 of the <span class="elsevierStyleItalic">COL7A1</span> gene, and the paternal mutation p.R1730X, found on exon 58 of the <span class="elsevierStyleItalic">COL7A1</span> gene. The patient's medical history included a previous pregnancy monitored at another hospital with threat of premature delivery at week 30 and cesarean section at week 34. The current pregnancy was spontaneous. The patient developed iron-deficiency anemia and vitamin D deficiency with poor oral tolerance in the first trimester, which required treatment with vitamin D supplement packets and 2 hospital stays for intravenous iron therapy. Cesarean section was performed at week 36<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>2 days due to vaginal stenosis; the infant girl weighed 2980<span class="elsevierStyleHsp" style=""></span>g at birth. The postsurgical period was without incident and the patient was able to start breastfeeding immediately after delivery (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">The diagnostic process for EB begins with an adequate medical history that includes relevant family history and a complete physical examination. The next step is to make an initial classification of the condition as one of the 4 main EB types by using immunofluorescence antigen mapping to identify the plane of cleavage. Once the affected protein is identified, genetic analysis can be carried out by sequencing the candidate gene.</p><p id="par0045" class="elsevierStylePara elsevierViewall">In both of our patients, mutations were found in the <span class="elsevierStyleItalic">COL7A1</span> gene; it was therefore possible to perform amniocentesis to reach a prenatal diagnosis in one of the women.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Genetic counseling and guidance are fundamental in these patients. Because RDEB is a recessive disease, prenatal or preimplantation diagnosis is only necessary if the patient's partner is suspected of being a carrier.</p><p id="par0055" class="elsevierStylePara elsevierViewall">No definitive cure for EB currently exists. Treatment of this disease is symptomatic and supportive, focusing primarily on preventing complications. Therapeutic approaches to RDEB aim to correct the deficiency or absence of specific anchor proteins in the dermoepidermal junction, with a particular focus on restoring expression of type VII collagen. Various protein-based, cellular, and genetic therapies are currently being investigated.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">3–5</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Although the literature on this subject is limited, the available evidence seems to indicate that RDEB increases the risk of maternal complications but does not contraindicate pregnancy.<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">6–11</span></a> In our experience, with strict monitoring made it possible to bring both of our patients’ pregnancies to term without major complications for mother or child. Early treatment of iron deficiency, vitamin deficiency, and skin infections are the cornerstone of pregnancy management in these patients. Although vaginal births without incident have been reported in pregnant women with RDEB, the type of delivery should be determined on an individual basis in accordance with the conditions of each patient.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">7,9,11</span></a> Scheduled cesarean section was performed in both of our patients due to vaginal stenosis. However, for patients without vaginal involvement, and in the absence of comorbidities that would contraindicate vaginal delivery (multiple previous cesarean sections, placenta previa, etc.), this could be an option to consider towards the end of pregnancy.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Conflicts of Interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres1133101" "titulo" => "Graphical abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:3 [ "identificador" => "xres1133102" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 2 => array:2 [ "identificador" => "xpalclavsec1065510" "titulo" => "Keywords" ] 3 => array:3 [ "identificador" => "xres1133103" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0015" ] ] ] 4 => array:2 [ "identificador" => "xpalclavsec1065511" "titulo" => "Palabras clave" ] 5 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 6 => array:3 [ "identificador" => "sec0010" "titulo" => "Case Descriptions" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0015" "titulo" => "Case 1" ] 1 => array:2 [ "identificador" => "sec0020" "titulo" => "Case 2" ] ] ] 7 => array:2 [ "identificador" => "sec0025" "titulo" => "Discussion" ] 8 => array:2 [ "identificador" => "sec0030" "titulo" => "Conflicts of Interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1065510" "palabras" => array:5 [ 0 => "Epidermolysis bullosa" 1 => "Pregnancy" 2 => "Female" 3 => "Pregnancy complications" 4 => "Dystrophic epidermolysis bullosa" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1065511" "palabras" => array:5 [ 0 => "Epidermólisis bullosa" 1 => "Embarazo" 2 => "Mujer" 3 => "Complicaciones del embarazo" 4 => "Epidermólisis bullosa distrófica" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Dystrophic epidermolysis bullosa is a rare inherited disease caused by mutations in the <span class="elsevierStyleItalic">COL7A1</span> gene. Its recessive variant (recessive dystrophic epidermolysis bullosa) is characterized by the absence or considerably reduced expression of type <span class="elsevierStyleSmallCaps">VII</span> 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.</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">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.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">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.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0015" class="elsevierStyleSection elsevierViewall"><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">La epidermólisis bullosa distrófica es una enfermedad hereditaria rara debida a mutaciones del gen <span class="elsevierStyleItalic">COL7A1</span>. Su variante recesiva (EBDR) se caracteriza por una marcada disminución o ausencia completa de colágeno tipo <span class="elsevierStyleSmallCaps">VII</span> (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.</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">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.</p><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">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.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Boria F, Maseda R, Martín-Cameán M, De la Calle M, de Lucas R. Epidermólisis bullosa distrófica recesiva y embarazo. Actas Dermosifiliogr. 2019;110:50–52.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 768 "Ancho" => 900 "Tamanyo" => 87703 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Patient 1. Erosions on the abdomen after cesarean section and pseudosyndactyly on both hands.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1105 "Ancho" => 900 "Tamanyo" => 84373 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Patient 2. Abdomen before cesarean section and partial anonychia.</p>" ] ] 2 => array:5 [ "identificador" => "fig0015" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => false "mostrarDisplay" => true "figura" => array:1 [ 0 => array:4 [ "imagen" => "fx1.jpeg" "Alto" => 514 "Ancho" => 1333 "Tamanyo" => 124950 ] ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib0060" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Extracutaneous manifestations and complications of inherited epidermolysis bullosa" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J.D. Fine" 1 => "J.E. Mellerio" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jaad.2009.03.053" "Revista" => array:6 [ "tituloSerie" => "J Am Acad Dermatol" "fecha" => "2009" "volumen" => "61" "paginaInicial" => "387" "paginaFinal" => "402" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19700011" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0065" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Inherited epidermolysis bullosa: Updated recommendations on diagnosis and classification" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J.D. Fine" 1 => "L. Bruckner-Tuderman" 2 => "R.A. Eady" 3 => "E.A. Bauer" 4 => "J.W. Bauer" 5 => "C. Has" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jaad.2014.01.903" "Revista" => array:6 [ "tituloSerie" => "J Am Acad Dermatol" "fecha" => "2014" "volumen" => "70" "paginaInicial" => "1103" "paginaFinal" => "1126" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24690439" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0070" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Innovative therapeutic strategies for recessive dystrophic epidermolysis bullosa" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "F. Larcher" 1 => "M. Del Río" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.ad.2015.01.007" "Revista" => array:6 [ "tituloSerie" => "Actas Dermosifiliogr" "fecha" => "2015" "volumen" => "106" "paginaInicial" => "376" "paginaFinal" => "382" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25796272" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0075" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Novel and emerging therapies in the treatment of recessive dystrophic epidermolysis bullosa" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "E. Rashidghamat" 1 => "J. McGrath" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.5582/irdr.2017.01005" "Revista" => array:6 [ "tituloSerie" => "Intractable Rare Dis Res" "fecha" => "2017" "volumen" => "6" "paginaInicial" => "6" "paginaFinal" => "20" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28357176" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0080" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Progress toward treatment and cure of epidermolysis bullosa: Summary of the DEBRA International Research Symposium EB2015" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J. Uitto" 1 => "L. Bruckner-Tuderman" 2 => "A. Christiano" 3 => "J. McGrath" 4 => "C. Has" 5 => "A. South" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jid.2015.10.050" "Revista" => array:6 [ "tituloSerie" => "J Invest Dermatol" "fecha" => "2016" "volumen" => "136" "paginaInicial" => "352" "paginaFinal" => "358" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26802230" "web" => "Medline" ] ] ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0085" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Cesarean delivery in a pregnant woman with mutilating recessive dystrophic epidermolysis bullosa" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "M. Turmo-Tejera" 1 => "J. García-Navia" 2 => "F. Suárez" 3 => "M. Echevarría-Moreno" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jclinane.2013.08.008" "Revista" => array:6 [ "tituloSerie" => "J Clin Anesth" "fecha" => "2014" "volumen" => "26" "paginaInicial" => "155" "paginaFinal" => "157" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24582181" "web" => "Medline" ] ] ] ] ] ] ] ] 6 => array:3 [ "identificador" => "bib0090" "etiqueta" => "7" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The course of pregnancy and childbirth in three mothers with recessive dystrophic epidermolysis bullosa" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "T. Hanafusa" 1 => "K. Tamai" 2 => "N. Umegaki" 3 => "Y. Yamaguchi" 4 => "S. Fukuda" 5 => "Y. Nishikawa" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1365-2230.2011.04179.x" "Revista" => array:6 [ "tituloSerie" => "Clin Exp Dermatol" "fecha" => "2012" "volumen" => "37" "paginaInicial" => "10" "paginaFinal" => "14" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22007850" "web" => "Medline" ] ] ] ] ] ] ] ] 7 => array:3 [ "identificador" => "bib0095" "etiqueta" => "8" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pregnancy and cesarean delivery in a patient with dystrophic epidermolysis bullosa" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "S. Bianca" 1 => "A. Reale" 2 => "G. Ettore" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Eur J Obstet Gynecol Reprode Biol" "fecha" => "2003" "volumen" => "110" "paginaInicial" => "235" "paginaFinal" => "236" ] ] ] ] ] ] 8 => array:3 [ "identificador" => "bib0100" "etiqueta" => "9" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Outcomes of 11 pregnancies in three patients with recessive forms of epidermolysis bullosa" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "S.D. Choi" 1 => "Y.C. Kho" 2 => "L.M. Rhodes" 3 => "G.K. Davis" 4 => "M.G. Chapman" 5 => "D.F. Murrell" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Br J Dermatol" "fecha" => "2011" "volumen" => "165" "paginaInicial" => "700" "paginaFinal" => "701" ] ] ] ] ] ] 9 => array:3 [ "identificador" => "bib0105" "etiqueta" => "10" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pregnancy and delivery in a patient with mutilating dystrophic epidermolysis bullosa (Hallopeau-Siemens type)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "U. Buscher" 1 => "J. Wessel" 2 => "I. Anton-Lamprecht" 3 => "J.W. Dudenhausen" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Obstet Gynecol" "fecha" => "1997" "volumen" => "89" "paginaInicial" => "817" "paginaFinal" => "820" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9166333" "web" => "Medline" ] ] ] ] ] ] ] ] 10 => array:3 [ "identificador" => "bib0110" "etiqueta" => "11" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A pregnancy complicated with fetal growth restriction in a patient with dystrophic epidermolysis bullosa" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "E. Ozkaya" 1 => "E. Baser" 2 => "G. Akgul" 3 => "T. Kucukozkan" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3109/01443615.2011.653595" "Revista" => array:6 [ "tituloSerie" => "J Obstet Gynaecol" "fecha" => "2012" "volumen" => "32" "paginaInicial" => "302" "paginaFinal" => "303" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22369411" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000011000000001/v1_201901030631/S1578219018304001/v1_201901030631/en/main.assets" "Apartado" => array:4 [ "identificador" => "76905" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Brief Comunications" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000011000000001/v1_201901030631/S1578219018304001/v1_201901030631/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219018304001?idApp=UINPBA000044" ]
año/Mes | Html | Total | |
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2024 Noviembre | 13 | 6 | 19 |
2024 Octubre | 107 | 64 | 171 |
2024 Septiembre | 101 | 42 | 143 |
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2023 Diciembre | 71 | 18 | 89 |
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2023 Octubre | 97 | 34 | 131 |
2023 Septiembre | 56 | 28 | 84 |
2023 Agosto | 43 | 19 | 62 |
2023 Julio | 62 | 40 | 102 |
2023 Junio | 55 | 26 | 81 |
2023 Mayo | 78 | 30 | 108 |
2023 Abril | 70 | 25 | 95 |
2023 Marzo | 74 | 30 | 104 |
2023 Febrero | 75 | 32 | 107 |
2023 Enero | 56 | 32 | 88 |
2022 Diciembre | 64 | 37 | 101 |
2022 Noviembre | 33 | 40 | 73 |
2022 Octubre | 40 | 34 | 74 |
2022 Septiembre | 74 | 37 | 111 |
2022 Agosto | 99 | 55 | 154 |
2022 Julio | 64 | 70 | 134 |
2022 Junio | 31 | 58 | 89 |
2022 Mayo | 98 | 41 | 139 |
2022 Abril | 151 | 37 | 188 |
2022 Marzo | 144 | 64 | 208 |
2022 Febrero | 154 | 36 | 190 |
2022 Enero | 182 | 52 | 234 |
2021 Diciembre | 128 | 46 | 174 |
2021 Noviembre | 125 | 47 | 172 |
2021 Octubre | 117 | 66 | 183 |
2021 Septiembre | 66 | 45 | 111 |
2021 Agosto | 92 | 28 | 120 |
2021 Julio | 74 | 28 | 102 |
2021 Junio | 63 | 40 | 103 |
2021 Mayo | 59 | 59 | 118 |
2021 Abril | 122 | 101 | 223 |
2021 Marzo | 74 | 50 | 124 |
2021 Febrero | 78 | 37 | 115 |
2021 Enero | 47 | 39 | 86 |
2020 Diciembre | 49 | 31 | 80 |
2020 Noviembre | 33 | 33 | 66 |
2020 Octubre | 24 | 11 | 35 |
2020 Septiembre | 44 | 22 | 66 |
2020 Agosto | 45 | 28 | 73 |
2020 Julio | 25 | 30 | 55 |
2020 Junio | 37 | 31 | 68 |
2020 Mayo | 53 | 11 | 64 |
2020 Abril | 14 | 12 | 26 |
2020 Marzo | 23 | 8 | 31 |
2020 Febrero | 2 | 0 | 2 |
2019 Mayo | 1 | 0 | 1 |
2019 Abril | 1 | 0 | 1 |
2019 Enero | 3 | 2 | 5 |