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        "resumen" => "<p class="elsevierStyleSimplePara elsevierViewall">Chronic infantile neurologic cutaneous articular &#40;CINCA&#41; syndrome is a serious chronic systemic inflammatory disease that presents at a young age and that is characterized by skin&#44; joint&#44; and central nervous system disease&#46; Skin symptoms are the first to appear&#44; in the form of a longstanding nonpruritic urticarial rash&#44; with exacerbations coinciding with episodes of fever&#44; arthritis&#44; and enlarged lymph nodes&#46; The findings of biopsy of skin lesions are extremely variable but characterized by perivascular neutrophilic infiltrate&#46; With the discovery of mutations in the <span class="elsevierStyleItalic">CIAS1</span> gene&#44; which encodes a protein known as cryopyrin&#44; this entity has been classified as one of the cryopyrin-associated autoinflammatory diseases&#44; along with familial cold urticaria and Muckle-Wells syndrome&#46; This discovery has also made available new therapeutic options&#46; We present the case of a boy diagnosed with CINCA syndrome who presented with an outbreak of painful skin lesions and fever&#46; These lesions were thought to be an exacerbation of underlying lesions during an episode of fever&#46;</p>"
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        "resumen" => "<p class="elsevierStyleSimplePara elsevierViewall">El s&#237;ndrome CINCA &#40;<span class="elsevierStyleItalic">Chronic Infantile Neurologic Cutaneous Articular Syndrome</span>&#41; es una grave enfermedad inflamatoria sist&#233;mica cr&#243;nica&#44; de instauraci&#243;n temprana&#44; que se caracteriza por afectaci&#243;n cut&#225;nea&#44; articular y del sistema nervioso central &#40;SNC&#41;&#46; La cl&#237;nica cut&#225;nea en forma de exantema urticariforme no pruriginoso es la primera en aparecer&#44; siendo m&#225;s o menos permanente con exacerbaciones que coinciden con brotes de fiebre&#44; artritis y adenopat&#237;as&#46; La biopsia de las lesiones cut&#225;neas es muy variable&#44; aunque destaca un infiltrado neutrof&#237;lico perivascular&#46; El descubrimiento de mutaciones en el gen CIAS1&#44; que codifica una prote&#237;na llamada criopirina&#44; ha permitido a&#241;adir esta entidad al espectro de las enfermedades autoinflamatorias asociadas a criopirinas&#44; junto con la urticaria familiar por fr&#237;o y el s&#237;ndrome de Muckle-Wells&#44; implicando adem&#225;s nuevas opciones terap&#233;uticas&#46; Presentamos el caso de un var&#243;n diagnosticado de s&#237;ndrome CINCA que present&#243; un brote de lesiones cut&#225;neas dolorosas y fiebre&#44; interpret&#225;ndose &#233;stas como una exacerbaci&#243;n de sus lesiones habituales durante un episodio febril&#46;</p>"
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Case reports
Exacerbation of Skin Lesions During Fever in a Patient With Chronic Infantile Neurologic Cutaneous Articular Syndrome
Síndrome Cinca: Presentación de un Caso Con Exacerbación Febril de Sus Lesiones Cutáneas
S. Aboín-Gonzáleza,
Corresponding author
asonsoles@hotmail.com

Correspondence: Servicio de Dermatología, Hospital Ramón y Cajal Universidad de Alcalá de Henares, Carretera de Colmenar Viejo, km 9,400, 28034 Madrid, Spain.
, I. Aldanondo-Fernández de la Moraa, C.R. García-Acebesa, R. Carrillo-Gijónb, A. Harto-Castañoa, P. Jaén-Olasoloa
a Servicio de Dermatología, Hospital Ramón y Cajal, Universidad de Alcalá de Henares, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital Ramón y Cajal, Universidad de Alcalá de Henares, Madrid, Spain
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      ]
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    "titulosAlternativos" => array:1 [
      "es" => array:1 [
        "titulo" => "S&#237;ndrome Cinca&#58; Presentaci&#243;n de un Caso Con Exacerbaci&#243;n Febril de Sus Lesiones Cut&#225;neas"
      ]
    ]
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    "fechaAceptado" => "2007-12-12"
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          "clase" => "keyword"
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            0 => "chronic infantile neurologic cutaneous and articular syndrome"
            1 => "neonatal onset multi-inflammatory disease"
            2 => "neutrophilic dermatitis"
            3 => "cryopyrin"
          ]
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          "palabras" => array:4 [
            0 => "CINCA &#40;<span class="elsevierStyleItalic">Chronic Infantile Neurologic Cutaneous Articular Syndrome</span>&#41;"
            1 => "NOMID &#40;<span class="elsevierStyleItalic">Neonatal Onset Multi-Inflammatory Disease</span>&#41;"
            2 => "dermatitis neutrof&#237;lica"
            3 => "criopirina"
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    "resumen" => array:2 [
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<p class="elsevierStyleSimplePara elsevierViewall">Chronic infantile neurologic cutaneous articular &#40;CINCA&#41; syndrome is a serious chronic systemic inflammatory disease that presents at a young age and that is characterized by skin&#44; joint&#44; and central nervous system disease&#46; Skin symptoms are the first to appear&#44; in the form of a longstanding nonpruritic urticarial rash&#44; with exacerbations coinciding with episodes of fever&#44; arthritis&#44; and enlarged lymph nodes&#46; The findings of biopsy of skin lesions are extremely variable but characterized by perivascular neutrophilic infiltrate&#46; With the discovery of mutations in the <span class="elsevierStyleItalic">CIAS1</span> gene&#44; which encodes a protein known as cryopyrin&#44; this entity has been classified as one of the cryopyrin-associated autoinflammatory diseases&#44; along with familial cold urticaria and Muckle-Wells syndrome&#46; This discovery has also made available new therapeutic options&#46; We present the case of a boy diagnosed with CINCA syndrome who presented with an outbreak of painful skin lesions and fever&#46; These lesions were thought to be an exacerbation of underlying lesions during an episode of fever&#46;</p>"
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      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<p class="elsevierStyleSimplePara elsevierViewall">El s&#237;ndrome CINCA &#40;<span class="elsevierStyleItalic">Chronic Infantile Neurologic Cutaneous Articular Syndrome</span>&#41; es una grave enfermedad inflamatoria sist&#233;mica cr&#243;nica&#44; de instauraci&#243;n temprana&#44; que se caracteriza por afectaci&#243;n cut&#225;nea&#44; articular y del sistema nervioso central &#40;SNC&#41;&#46; La cl&#237;nica cut&#225;nea en forma de exantema urticariforme no pruriginoso es la primera en aparecer&#44; siendo m&#225;s o menos permanente con exacerbaciones que coinciden con brotes de fiebre&#44; artritis y adenopat&#237;as&#46; La biopsia de las lesiones cut&#225;neas es muy variable&#44; aunque destaca un infiltrado neutrof&#237;lico perivascular&#46; El descubrimiento de mutaciones en el gen CIAS1&#44; que codifica una prote&#237;na llamada criopirina&#44; ha permitido a&#241;adir esta entidad al espectro de las enfermedades autoinflamatorias asociadas a criopirinas&#44; junto con la urticaria familiar por fr&#237;o y el s&#237;ndrome de Muckle-Wells&#44; implicando adem&#225;s nuevas opciones terap&#233;uticas&#46; Presentamos el caso de un var&#243;n diagnosticado de s&#237;ndrome CINCA que present&#243; un brote de lesiones cut&#225;neas dolorosas y fiebre&#44; interpret&#225;ndose &#233;stas como una exacerbaci&#243;n de sus lesiones habituales durante un episodio febril&#46;</p>"
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Article information
ISSN: 15782190
Original language: English
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Idiomas
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