array:23 [
  "pii" => "S1578219018301914"
  "issn" => "15782190"
  "doi" => "10.1016/j.adengl.2018.05.030"
  "estado" => "S300"
  "fechaPublicacion" => "2019-01-01"
  "aid" => "1943"
  "copyright" => "Elsevier España, S.L.U. and AEDV"
  "copyrightAnyo" => "2018"
  "documento" => "simple-article"
  "crossmark" => 1
  "subdocumento" => "crp"
  "cita" => "Actas Dermosifiliogr. 2019;110:81-3"
  "abierto" => array:3 [
    "ES" => true
    "ES2" => true
    "LATM" => true
  ]
  "gratuito" => true
  "lecturas" => array:2 [
    "total" => 8
    "formatos" => array:2 [
      "HTML" => 7
      "PDF" => 1
    ]
  ]
  "Traduccion" => array:1 [
    "es" => array:19 [
      "pii" => "S0001731018301571"
      "issn" => "00017310"
      "doi" => "10.1016/j.ad.2017.10.025"
      "estado" => "S300"
      "fechaPublicacion" => "2019-01-01"
      "aid" => "1943"
      "copyright" => "AEDV"
      "documento" => "simple-article"
      "crossmark" => 1
      "subdocumento" => "crp"
      "cita" => "Actas Dermosifiliogr. 2019;110:81-3"
      "abierto" => array:3 [
        "ES" => true
        "ES2" => true
        "LATM" => true
      ]
      "gratuito" => true
      "lecturas" => array:2 [
        "total" => 177
        "formatos" => array:2 [
          "HTML" => 58
          "PDF" => 119
        ]
      ]
      "es" => array:11 [
        "idiomaDefecto" => true
        "cabecera" => "<span class="elsevierStyleTextfn">CARTA CIENT&#205;FICO-CL&#205;NICA</span>"
        "titulo" => "Miocardiopat&#237;a dilatada en una ni&#241;a con epiderm&#243;lisis ampollar distr&#243;fica recesiva"
        "tienePdf" => "es"
        "tieneTextoCompleto" => "es"
        "paginas" => array:1 [
          0 => array:2 [
            "paginaInicial" => "81"
            "paginaFinal" => "83"
          ]
        ]
        "titulosAlternativos" => array:1 [
          "en" => array:1 [
            "titulo" => "Dilated Cardiomyopathy in a Child with Recessive Dystrophic Epidermolysis Bullosa"
          ]
        ]
        "contieneTextoCompleto" => array:1 [
          "es" => true
        ]
        "contienePdf" => array:1 [
          "es" => true
        ]
        "resumenGrafico" => array:2 [
          "original" => 0
          "multimedia" => array:7 [
            "identificador" => "fig0005"
            "etiqueta" => "Figura 1"
            "tipo" => "MULTIMEDIAFIGURA"
            "mostrarFloat" => true
            "mostrarDisplay" => false
            "figura" => array:1 [
              0 => array:4 [
                "imagen" => "gr1.jpeg"
                "Alto" => 1321
                "Ancho" => 850
                "Tamanyo" => 124588
              ]
            ]
            "descripcion" => array:1 [
              "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Erosiones generalizadas en el tronco y gastrostom&#237;a&#46;</p>"
            ]
          ]
        ]
        "autores" => array:1 [
          0 => array:2 [
            "autoresLista" => "A&#46; Imbern&#243;n-Moya, R&#46; Maseda-Pedrero, M&#46; Feito, R&#46; de Lucas"
            "autores" => array:4 [
              0 => array:2 [
                "nombre" => "A&#46;"
                "apellidos" => "Imbern&#243;n-Moya"
              ]
              1 => array:2 [
                "nombre" => "R&#46;"
                "apellidos" => "Maseda-Pedrero"
              ]
              2 => array:2 [
                "nombre" => "M&#46;"
                "apellidos" => "Feito"
              ]
              3 => array:2 [
                "nombre" => "R&#46;"
                "apellidos" => "de Lucas"
              ]
            ]
          ]
        ]
      ]
      "idiomaDefecto" => "es"
      "Traduccion" => array:1 [
        "en" => array:9 [
          "pii" => "S1578219018301914"
          "doi" => "10.1016/j.adengl.2018.05.030"
          "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/S1578219018301914?idApp=UINPBA000044"
        ]
      ]
      "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731018301571?idApp=UINPBA000044"
      "url" => "/00017310/0000011000000001/v1_201901030610/S0001731018301571/v1_201901030610/es/main.assets"
    ]
  ]
  "itemAnterior" => array:19 [
    "pii" => "S1578219018304153"
    "issn" => "15782190"
    "doi" => "10.1016/j.adengl.2018.11.017"
    "estado" => "S300"
    "fechaPublicacion" => "2019-01-01"
    "aid" => "1926"
    "copyright" => "Elsevier Espa&#241;a&#44; S&#46;L&#46;U&#46; and AEDV"
    "documento" => "simple-article"
    "crossmark" => 1
    "subdocumento" => "crp"
    "cita" => "Actas Dermosifiliogr. 2019;110:78-81"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:2 [
      "total" => 4
      "formatos" => array:2 [
        "HTML" => 2
        "PDF" => 2
      ]
    ]
    "en" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Case and Research Letters</span>"
      "titulo" => "Progesterone Autoimmune Dermatitis Responding to Ulipristal Acetate"
      "tienePdf" => "en"
      "tieneTextoCompleto" => "en"
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "78"
          "paginaFinal" => "81"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "es" => array:1 [
          "titulo" => "Respuesta de la dermatitis autoinmune por progesterona al acetato de ulipristal"
        ]
      ]
      "contieneTextoCompleto" => array:1 [
        "en" => true
      ]
      "contienePdf" => array:1 [
        "en" => true
      ]
      "resumenGrafico" => array:2 [
        "original" => 0
        "multimedia" => array:7 [
          "identificador" => "fig0005"
          "etiqueta" => "Figure 1"
          "tipo" => "MULTIMEDIAFIGURA"
          "mostrarFloat" => true
          "mostrarDisplay" => false
          "figura" => array:1 [
            0 => array:4 [
              "imagen" => "gr1.jpeg"
              "Alto" => 1822
              "Ancho" => 1200
              "Tamanyo" => 232392
            ]
          ]
          "descripcion" => array:1 [
            "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A&#44; Erythematous&#44; edematous plaque on the right forearm that is tender and infiltrated to touch measuring some 10<span class="elsevierStyleHsp" style=""></span>cm in diameter&#46; Patch test with progesterone&#44; which was negative at 96<span class="elsevierStyleHsp" style=""></span>hours&#44; and with NorLevo in petrolatum and Progeffik applied directly on the area of the lesion and on healthy skin &#40;B&#41;&#46;</p>"
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "S&#46; Oscoz-Jaime, M&#46; Larrea-Garc&#237;a, M&#46;J&#46; Mitxelena-Eceiza, N&#46; Abi&#225;n-Franco"
          "autores" => array:4 [
            0 => array:2 [
              "nombre" => "S&#46;"
              "apellidos" => "Oscoz-Jaime"
            ]
            1 => array:2 [
              "nombre" => "M&#46;"
              "apellidos" => "Larrea-Garc&#237;a"
            ]
            2 => array:2 [
              "nombre" => "M&#46;J&#46;"
              "apellidos" => "Mitxelena-Eceiza"
            ]
            3 => array:2 [
              "nombre" => "N&#46;"
              "apellidos" => "Abi&#225;n-Franco"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "en"
    "Traduccion" => array:1 [
      "es" => array:9 [
        "pii" => "S0001731018300887"
        "doi" => "10.1016/j.ad.2017.11.016"
        "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/S0001731018300887?idApp=UINPBA000044"
      ]
    ]
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219018304153?idApp=UINPBA000044"
    "url" => "/15782190/0000011000000001/v1_201901030631/S1578219018304153/v1_201901030631/en/main.assets"
  ]
  "en" => array:16 [
    "idiomaDefecto" => true
    "cabecera" => "<span class="elsevierStyleTextfn">Case and Research Letters</span>"
    "titulo" => "Dilated Cardiomyopathy in a Child with Recessive Dystrophic Epidermolysis Bullosa"
    "tieneTextoCompleto" => true
    "saludo" => "<span class="elsevierStyleItalic">To the Editor&#58;</span>"
    "paginas" => array:1 [
      0 => array:2 [
        "paginaInicial" => "81"
        "paginaFinal" => "83"
      ]
    ]
    "autores" => array:1 [
      0 => array:4 [
        "autoresLista" => "A&#46; Imbern&#243;n-Moya, R&#46; Maseda-Pedrero, M&#46; Feito, R&#46; de Lucas"
        "autores" => array:4 [
          0 => array:4 [
            "nombre" => "A&#46;"
            "apellidos" => "Imbern&#243;n-Moya"
            "email" => array:1 [
              0 => "adrian&#95;imber88&#64;hotmail&#46;com"
            ]
            "referencia" => array:2 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">a</span>"
                "identificador" => "aff0005"
              ]
              1 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">&#42;</span>"
                "identificador" => "cor0005"
              ]
            ]
          ]
          1 => array:3 [
            "nombre" => "R&#46;"
            "apellidos" => "Maseda-Pedrero"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">b</span>"
                "identificador" => "aff0010"
              ]
            ]
          ]
          2 => array:3 [
            "nombre" => "M&#46;"
            "apellidos" => "Feito"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">b</span>"
                "identificador" => "aff0010"
              ]
            ]
          ]
          3 => array:3 [
            "nombre" => "R&#46;"
            "apellidos" => "de Lucas"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">b</span>"
                "identificador" => "aff0010"
              ]
            ]
          ]
        ]
        "afiliaciones" => array:2 [
          0 => array:3 [
            "entidad" => "Departamento de Dermatolog&#237;a&#44; Hospital Universitario Severo Ochoa&#44; Legan&#233;s&#44; Madrid&#44; Spain"
            "etiqueta" => "a"
            "identificador" => "aff0005"
          ]
          1 => array:3 [
            "entidad" => "Departamento de Dermatolog&#237;a&#44; Hospital Universitario La Paz&#44; Madrid&#44; Spain"
            "etiqueta" => "b"
            "identificador" => "aff0010"
          ]
        ]
        "correspondencia" => array:1 [
          0 => array:3 [
            "identificador" => "cor0005"
            "etiqueta" => "&#8270;"
            "correspondencia" => "Corresponding author&#46;"
          ]
        ]
      ]
    ]
    "titulosAlternativos" => array:1 [
      "es" => array:1 [
        "titulo" => "Miocardiopat&#237;a dilatada en una ni&#241;a con epiderm&#243;lisis ampollar distr&#243;fica recesiva"
      ]
    ]
    "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" => 1275
            "Ancho" => 850
            "Tamanyo" => 131638
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">generalized erosions on her trunk&#46;</p>"
        ]
      ]
    ]
    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Dilated cardiomyopathy &#40;DC&#41; is a progressive dilatation and impaired contractility of the left or both ventricles&#46; Predisposing factors may involve familial&#47;genetic&#44; viral infection&#44; autoimmune&#44; nutritional deficit&#44; iron overload&#44; chronic anaemia and drugs&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Sharratt et al&#46; reported in 1986 the first case of DC in a patient with epidermolysis bullosa &#40;EB&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> Since then&#44; the clinical association of EB and DC has been described in several case reports and case series&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#8211;6</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">A</span> 6-year-old child with severe generalized recessive dystrophic epidermolysis bullosa &#40;RDEB&#41;&#46; Treatment with daily cures and moisturizing was made&#46; Her brother&#44; her twin sister and her parents had no relevant medical history&#46; The patient had enteral nutrition by a gastrostomy since 2 years ago&#46; She had chronic anaemia treated with intravenous iron and periodic transfusions were required&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was referred to the Emergency Department due to respiratory distress and influenza-like syndrome for four days&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Her general condition was bad&#46; Her temperature was 36&#46;4<span class="elsevierStyleHsp" style=""></span>&#176;C&#44; blood pressure 100&#47;50<span class="elsevierStyleHsp" style=""></span>mmHg&#44; heart rate 125 bpm&#44; and oxygen saturation of 94&#37; with room air&#46; Cutaneous examination showed generalized erosions and syndactalia on her hands with a severe functional limitation &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figures 1&#44; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Electrocardiogram presented diffuse changes in repolarization&#46; Chest radiograph revealed the presence of cardiomegaly and acute pulmonary oedema &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Echocardiogram showed severely dilated left ventricle with ejection fraction of 40&#37;&#44; mild tricuspid insufficiency and moderate pulmonary insufficiency&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The biochemical parameters showed glucose 113<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; C-reactive protein 120<span class="elsevierStyleHsp" style=""></span>mg&#47;L&#44; total proteins 9&#46;8<span class="elsevierStyleHsp" style=""></span>g&#47;dL&#44; haemoglobin 9&#46;2<span class="elsevierStyleHsp" style=""></span>g&#47;dL&#44; mean corpuscular volume 91&#46;4 fl&#44; 578000 platelets&#47;mm<span class="elsevierStyleSup">3</span>&#44; 15300 leucocytes&#47;mm<span class="elsevierStyleSup">3</span> &#40;polymorphonuclear leucocytes 72&#46;6&#37;&#44; lymphocytes 17&#46;4&#37;&#44; monocytes 7&#46;8&#37;&#44; eosinophils 2&#46;1&#37;&#44; basophils 0&#46;1&#37;&#41;&#46; Blood cultures were negative&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The patient was admitted to the Intensive Care Unit with a diagnosis of DC&#44; congestive heart failure&#44; acute pulmonary oedema and cardiogenic shock&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Treatment with high flow oxygen therapy&#44; non-invasive mechanical ventilation&#44; hydrochlorothiazide&#44; spironolactone&#44; enalapril&#44; carvedilol and aspirine was initiated&#46; The patient presented clinical improvement after 2 months of treatment&#46; However&#44; she had progressive worsening and was followed up by the palliative care unit&#46; Sshe died due to multiple organ failure nine months later&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Discussion</span><p id="par0055" class="elsevierStylePara elsevierViewall">Multiple structural and functional alterations in the echocardiogram have been reported in RDEB patients including dilated aortic root&#44; left ventricular hypertrophy&#44; dilatation and dysfunction&#44; increased left ventricular mass and depressed right ventricular systolic function&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">DC usually appears in cases of severe generalized RDEB&#46; The most common EB subtypes associated with DC are RDEB and junctional EB-non-Herlitz&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The age at diagnosis of DC in RDEB ranges from 2 to 28 years&#44; with a variable ejection fraction from 9 to 45&#37;&#46; Mortality rate varies from 30-60&#37; and death usually occurs within the first 3 months after diagnosis of DC&#46; DC is usually detected at an advanced stage with low ejection fraction&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#8211;6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Development of DC in RDEB is due to multifactorial disorders&#46; Contributor factors may include chronic anaemia&#44; iron overload&#44; poor nutritional status&#44; albumin and carnitine low levels&#44; selenium and zinc deficiency&#44; cardiotoxic drugs&#44; viral infections&#44; inflammatory conditions&#44; structural defects in myocardium&#44; chronic renal insufficiency and hypoaminoacedemia&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a> To date there is no evidence of mutation in type VII collagen or the basement membrane zone in the pathogenesis of DC&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">History of prior viral infection is usually associated with a more favourable outcome&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> We suggest that our patient presented DC due mainly to chronic anaemia&#44; iron overload and nutritional deficiency&#46; Viral infection of the upper respiratory tract was the trigger for the development of heart failure and death&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">In conclusion&#44; a RDEB patient who develops influenza-like illness and fever requires an urgent cardiac evaluation including electrocardiogram&#44; chest radiograph and echocardiogram&#46; Screening laboratory evaluations are also necessary including albumin&#44; carnitine&#44; selenium&#44; zinc and haemoglobin levels and viruses serology&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">5&#44;6</span></a> A complete drug history is essential&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> An early diagnosis and treatment can delay clinical progression and reduce morbidity and mortality&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflicto de intereses</span><p id="par0085" class="elsevierStylePara elsevierViewall">Los autores declaran no tener ning&#250;n conflicto de intereses&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
      "secciones" => array:3 [
        0 => array:2 [
          "identificador" => "sec0005"
          "titulo" => "Discussion"
        ]
        1 => array:2 [
          "identificador" => "sec0010"
          "titulo" => "Conflicto de intereses"
        ]
        2 => array:1 [
          "titulo" => "Bibliograf&#237;a"
        ]
      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "NotaPie" => array:1 [
      0 => array:2 [
        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Imbern&#243;n-Moya A&#44; Maseda-Pedrero R&#44; Feito M&#44; de Lucas R&#46; Miocardiopat&#237;a dilatada en una ni&#241;a con epiderm&#243;lisis ampollar distr&#243;fica recesiva&#46; Actas Dermosifiliogr&#46; 2019&#59;110&#58;81&#8211;83&#46;</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" => 1321
            "Ancho" => 850
            "Tamanyo" => 124588
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">generalized erosions on her trunk and gastrostomy&#46;</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" => 1275
            "Ancho" => 850
            "Tamanyo" => 131638
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">generalized erosions on her trunk&#46;</p>"
        ]
      ]
      2 => array:7 [
        "identificador" => "fig0015"
        "etiqueta" => "Figure 3"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr3.jpeg"
            "Alto" => 770
            "Ancho" => 900
            "Tamanyo" => 49403
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">cardiomegaly and findings of acute pulmonary oedema&#46;</p>"
        ]
      ]
    ]
    "bibliografia" => array:2 [
      "titulo" => "Bibliograf&#237;a"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:6 [
            0 => array:3 [
              "identificador" => "bib0035"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Dilated cardiomyopathy in children"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "M&#46;G&#46; Gagliardi"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1080/08035320600649358"
                      "Revista" => array:6 [
                        "tituloSerie" => "Acta Paediatr Suppl&#46;"
                        "fecha" => "2006"
                        "volumen" => "95"
                        "paginaInicial" => "14"
                        "paginaFinal" => "16"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16801159"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0040"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Echocardiography of intracardiac filling defects in infants and children"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "G&#46;P&#46; Sharratt"
                            1 => "A&#46;G&#46; Lacson"
                            2 => "G&#46; Cornel"
                            3 => "S&#46; Virmani"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Pediatr Cardiol&#46;"
                        "fecha" => "1986"
                        "volumen" => "7"
                        "paginaInicial" => "189"
                        "paginaFinal" => "194"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0045"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Ventricular dysfunction and aortic dilation in patients with recessive dystrophic epidermolysis bullosa"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "T&#46;D&#46; Ryan"
                            1 => "A&#46;W&#46; Lucky"
                            2 => "E&#46;C&#46; King"
                            3 => "G&#46; Huang"
                            4 => "J&#46;A&#46; Towbin"
                            5 => "J&#46;L&#46; Jefferies"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1111/bjd.14168"
                      "Revista" => array:6 [
                        "tituloSerie" => "Br J Dermatol&#46;"
                        "fecha" => "2016"
                        "volumen" => "174"
                        "paginaInicial" => "671"
                        "paginaFinal" => "673"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26370777"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0050"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Dilated cardiomyopathy in epidermolysis bullosa&#58; A retrospective&#44; multicenter study"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "I&#46; Lara-Corrales"
                            1 => "J&#46;E&#46; Mellerio"
                            2 => "A&#46;E&#46; Martinez"
                            3 => "A&#46; Greeen"
                            4 => "A&#46;W&#46; Lucky"
                            5 => "R&#46;G&#46; Azizkhan"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1111/j.1525-1470.2010.01127.x"
                      "Revista" => array:6 [
                        "tituloSerie" => "Pediatr Dermatol"
                        "fecha" => "2010"
                        "volumen" => "27"
                        "paginaInicial" => "238"
                        "paginaFinal" => "243"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20609141"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib0055"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Dilated cardiomyopathy in epidermolysis bullosa"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "I&#46; Lara-Corrales"
                            1 => "E&#46; Pope"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.det.2010.02.002"
                      "Revista" => array:6 [
                        "tituloSerie" => "Dermatol Clin&#46;"
                        "fecha" => "2010"
                        "volumen" => "28"
                        "paginaInicial" => "347"
                        "paginaFinal" => "351"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20447501"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib0060"
              "etiqueta" => "6"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Cardiomyopathy in patients with hereditary bullous epidermolysis"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "A&#46; Batalla"
                            1 => "A&#46; Vicente"
                            2 => "J&#46; Bartrons"
                            3 => "F&#46; Prada"
                            4 => "C&#46; Fortuny"
                            5 => "M&#46;A&#46; Gonz&#225;lez-Ense&#241;at"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.ad.2017.01.015"
                      "Revista" => array:6 [
                        "tituloSerie" => "Actas Dermosifiliogr&#46;"
                        "fecha" => "2017"
                        "volumen" => "108"
                        "paginaInicial" => "544"
                        "paginaFinal" => "549"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28364942"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
  ]
  "idiomaDefecto" => "en"
  "url" => "/15782190/0000011000000001/v1_201901030631/S1578219018301914/v1_201901030631/en/main.assets"
  "Apartado" => array:4 [
    "identificador" => "6157"
    "tipo" => "SECCION"
    "en" => array:2 [
      "titulo" => "Case and Research Letters"
      "idiomaDefecto" => true
    ]
    "idiomaDefecto" => "en"
  ]
  "PDF" => "https://static.elsevier.es/multimedia/15782190/0000011000000001/v1_201901030631/S1578219018301914/v1_201901030631/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/"
  "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219018301914?idApp=UINPBA000044"
]
Compartir
Información de la revista
Vol. 110. Núm. 1.
Páginas 81-83 (enero - febrero 2019)
Vol. 110. Núm. 1.
Páginas 81-83 (enero - febrero 2019)
Case and Research Letters
Acceso a texto completo
Dilated Cardiomyopathy in a Child with Recessive Dystrophic Epidermolysis Bullosa
Miocardiopatía dilatada en una niña con epidermólisis ampollar distrófica recesiva
Visitas
4755
A. Imbernón-Moyaa,
Autor para correspondencia
adrian_imber88@hotmail.com

Corresponding author.
, R. Maseda-Pedrerob, M. Feitob, R. de Lucasb
a Departamento de Dermatología, Hospital Universitario Severo Ochoa, Leganés, Madrid, Spain
b Departamento de Dermatología, Hospital Universitario La Paz, Madrid, Spain
Este artículo ha recibido
Información del artículo
Texto completo
Bibliografía
Descargar PDF
Estadísticas
Figuras (3)
Mostrar másMostrar menos
Texto completo
To the Editor:

Dilated cardiomyopathy (DC) is a progressive dilatation and impaired contractility of the left or both ventricles. Predisposing factors may involve familial/genetic, viral infection, autoimmune, nutritional deficit, iron overload, chronic anaemia and drugs.1

Sharratt et al. reported in 1986 the first case of DC in a patient with epidermolysis bullosa (EB).2 Since then, the clinical association of EB and DC has been described in several case reports and case series.2–6

A 6-year-old child with severe generalized recessive dystrophic epidermolysis bullosa (RDEB). Treatment with daily cures and moisturizing was made. Her brother, her twin sister and her parents had no relevant medical history. The patient had enteral nutrition by a gastrostomy since 2 years ago. She had chronic anaemia treated with intravenous iron and periodic transfusions were required.

The patient was referred to the Emergency Department due to respiratory distress and influenza-like syndrome for four days.

Her general condition was bad. Her temperature was 36.4°C, blood pressure 100/50mmHg, heart rate 125 bpm, and oxygen saturation of 94% with room air. Cutaneous examination showed generalized erosions and syndactalia on her hands with a severe functional limitation (Figures 1, 2).

Figure 1.

generalized erosions on her trunk and gastrostomy.

(0.12MB).
Figure 2.

generalized erosions on her trunk.

(0.13MB).

Electrocardiogram presented diffuse changes in repolarization. Chest radiograph revealed the presence of cardiomegaly and acute pulmonary oedema (Figure 3).

Figure 3.

cardiomegaly and findings of acute pulmonary oedema.

(0.05MB).

Echocardiogram showed severely dilated left ventricle with ejection fraction of 40%, mild tricuspid insufficiency and moderate pulmonary insufficiency.

The biochemical parameters showed glucose 113mg/dL, C-reactive protein 120mg/L, total proteins 9.8g/dL, haemoglobin 9.2g/dL, mean corpuscular volume 91.4 fl, 578000 platelets/mm3, 15300 leucocytes/mm3 (polymorphonuclear leucocytes 72.6%, lymphocytes 17.4%, monocytes 7.8%, eosinophils 2.1%, basophils 0.1%). Blood cultures were negative.

The patient was admitted to the Intensive Care Unit with a diagnosis of DC, congestive heart failure, acute pulmonary oedema and cardiogenic shock.

Treatment with high flow oxygen therapy, non-invasive mechanical ventilation, hydrochlorothiazide, spironolactone, enalapril, carvedilol and aspirine was initiated. The patient presented clinical improvement after 2 months of treatment. However, she had progressive worsening and was followed up by the palliative care unit. Sshe died due to multiple organ failure nine months later.

Discussion

Multiple structural and functional alterations in the echocardiogram have been reported in RDEB patients including dilated aortic root, left ventricular hypertrophy, dilatation and dysfunction, increased left ventricular mass and depressed right ventricular systolic function.3

DC usually appears in cases of severe generalized RDEB. The most common EB subtypes associated with DC are RDEB and junctional EB-non-Herlitz.4–6

The age at diagnosis of DC in RDEB ranges from 2 to 28 years, with a variable ejection fraction from 9 to 45%. Mortality rate varies from 30-60% and death usually occurs within the first 3 months after diagnosis of DC. DC is usually detected at an advanced stage with low ejection fraction.2–6

Development of DC in RDEB is due to multifactorial disorders. Contributor factors may include chronic anaemia, iron overload, poor nutritional status, albumin and carnitine low levels, selenium and zinc deficiency, cardiotoxic drugs, viral infections, inflammatory conditions, structural defects in myocardium, chronic renal insufficiency and hypoaminoacedemia.4–6 To date there is no evidence of mutation in type VII collagen or the basement membrane zone in the pathogenesis of DC.5

History of prior viral infection is usually associated with a more favourable outcome.4 We suggest that our patient presented DC due mainly to chronic anaemia, iron overload and nutritional deficiency. Viral infection of the upper respiratory tract was the trigger for the development of heart failure and death.

In conclusion, a RDEB patient who develops influenza-like illness and fever requires an urgent cardiac evaluation including electrocardiogram, chest radiograph and echocardiogram. Screening laboratory evaluations are also necessary including albumin, carnitine, selenium, zinc and haemoglobin levels and viruses serology.5,6 A complete drug history is essential.4 An early diagnosis and treatment can delay clinical progression and reduce morbidity and mortality.4–6

Conflicto de intereses

Los autores declaran no tener ningún conflicto de intereses.

Bibliografía
[1]
M.G. Gagliardi.
Dilated cardiomyopathy in children.
Acta Paediatr Suppl., 95 (2006), pp. 14-16
[2]
G.P. Sharratt, A.G. Lacson, G. Cornel, S. Virmani.
Echocardiography of intracardiac filling defects in infants and children.
Pediatr Cardiol., 7 (1986), pp. 189-194
[3]
T.D. Ryan, A.W. Lucky, E.C. King, G. Huang, J.A. Towbin, J.L. Jefferies.
Ventricular dysfunction and aortic dilation in patients with recessive dystrophic epidermolysis bullosa.
Br J Dermatol., 174 (2016), pp. 671-673
[4]
I. Lara-Corrales, J.E. Mellerio, A.E. Martinez, A. Greeen, A.W. Lucky, R.G. Azizkhan, et al.
Dilated cardiomyopathy in epidermolysis bullosa: A retrospective, multicenter study.
Pediatr Dermatol, 27 (2010), pp. 238-243
[5]
I. Lara-Corrales, E. Pope.
Dilated cardiomyopathy in epidermolysis bullosa.
Dermatol Clin., 28 (2010), pp. 347-351
[6]
A. Batalla, A. Vicente, J. Bartrons, F. Prada, C. Fortuny, M.A. González-Enseñat.
Cardiomyopathy in patients with hereditary bullous epidermolysis.
Actas Dermosifiliogr., 108 (2017), pp. 544-549

Please cite this article as: Imbernón-Moya A, Maseda-Pedrero R, Feito M, de Lucas R. Miocardiopatía dilatada en una niña con epidermólisis ampollar distrófica recesiva. Actas Dermosifiliogr. 2019;110:81–83.

Copyright © 2018. Elsevier España, S.L.U. and AEDV
Descargar PDF
Idiomas
Actas Dermo-Sifiliográficas
Opciones de artículo
Herramientas
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?