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Stevens–Johnson syndrome in childhood
Síndrome de Stevens-Johnson en la infancia
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"apellidos" => "Torrelo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Oftalmología, Hospital Infantil Universitario Niño Jesús, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Infantil Universitario Niño Jesús, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Stevens-Johnson en la infancia" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1130 "Ancho" => 750 "Tamanyo" => 128127 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Generalized exanthema with de-epithelized areas and Nyckolsky<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>(upper arrow), crusts (middle arrow) and bullous areas (lower arrow).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">The Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are parts of a single clinic spectrum in the context of the bullous mucocutaneous syndrome, and are differentiated by the degree of cutaneous involvement, which is below 10% in SJS and above 30% in TEN. In addition, at least 2 mucous membrane are usually affected.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–4</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Clinic case reports</span><p id="par0010" class="elsevierStylePara elsevierViewall">An 8 year-old boy with laryngeal cough, conjunctivitis (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>), oral mucosa erythema (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), peeling in the gland and fever, admitted to the pediatric intensive care unit (PICU) with suspected atypical SJS. Positive serology was obtained for Mycoplasma pneumoniae, which required the administration of clarithromycin and oral prednisone.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">At the ophthalmological level, the patient was assessed daily and given intensive ocular treatment consisting in cleaning of pseudo-membranes, hourly hydration, broad range antibiotic every 8<span class="elsevierStyleHsp" style=""></span>h as a prophylactic measure due to corneal de-epithelization, topical corticoids every 4<span class="elsevierStyleHsp" style=""></span>h and autogenous serum.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Sixteen days after admission, the patient was released without ophthalmological or systemic sequels.</p><p id="par0025" class="elsevierStylePara elsevierViewall">A 10-year-old girl who was administered ibuprofen due to fever and in the course of 10 days developed bilateral conjunctival erythema, oral mucosa erythema, macular exanthema in the torso (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>) and high fever. She was admitted to the PICU. The lesions extended progressively affecting 50% of bodily surface, requiring administration of corticoids and intravenous immunoglobulin.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Ocular involvement progressed to pseudomembranous conjunctivitis and corneal ulcers (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>). Accordingly, intensive ocular treatment was applied, subsequently requiring amniotic membrane graft in the left eye. One month after admission, the patient exhibited severe dry eye syndrome that progressed to keratinization with free edges, distichysiasis, limbar insufficiency, peripheral corneal vascularization in both eyes that required intrastromal and subconjunctival injection of bevacizumab, leukoma in the left eye involving the visual axis, intense photophobia and diminished visual acuity.</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">A 5-year-old girl with fever was treated with ibuprofen and paracetamol. After 48<span class="elsevierStyleHsp" style=""></span>h of treatment, fever persisted and she exhibited edema in the eyelids and lips as well as macular exanthema in the torso, for which reason she was admitted to the PICU and given treatment with corticoids, intravenous immunoglobulin and cyclosporine. Despite the treatment, the condition progressed to cover 100% of the skin. Intensive ocular treatment was established due to corneal de-epithelialization, adding reinforced antibiotic therapy and voriconazole eyedrops due to growth of Fusarium on facial skin.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Three weeks later, the right eye ulcer exhibited thinning and hypopion level in anterior chamber (<a class="elsevierStyleCrossRef" href="#fig0025">Fig. 5</a>). Evolution was torpid and at one month the patient exhibited ocular perforation (<a class="elsevierStyleCrossRef" href="#fig0030">Fig. 6</a>). Due to the severity of the condition, it was decided to enucleate the eye of the patient. Two months later, the left eye exhibited severe dry eye syndrome evolving to keratinization with free edges and ectropion.</p><elsevierMultimedia ident="fig0025"></elsevierMultimedia><elsevierMultimedia ident="fig0030"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">SJS and TEN produce significant and devastating sequels in survivors. Immunological mechanisms are involved in their expression and it is known that the most frequently related trigger is a medicament.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> Mycoplasma remains controversial as a cause of atypical SJS, with more latent systemic and ocular involvement and very few cutaneous lesions if any, as in the case of the present patient and as described in other published articles.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,5,6</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Frequently, the ocular surface is affected in the acute stages of said disease (50–81%), leading to the appearance of late, multifactorial signs that appear in up to 30–50% of patients.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3,7,8</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">There is no evidence that systemic treatments with high doses of corticoids and intravenous immunoglobulin is beneficial in the course of the ocular disease.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2,8</span></a> Even though there is no standard ocular treatment, new data recommend early treatment in the acute stage is to avoid sequels as much as possible.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3,7,9</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Daily ocular examination is required to pay special attention to the skin of the eyelids, the free edges thereof, the conjunctiva and the corneal surface. Amniotic membrane graft in the cases with poor prognosis and in the first days of evolution has demonstrated to be efficient, diminishing inflammation and attenuating the risk of developing visual sequelss, as in the case of the second patient who presented persistent epithelial defects in the left eye. Said treatments achieved the closure of said defects and the absence of new de-epithelialization or corneal ulcerations. These results agree with those published by Darren, according to which 10 out of 28 examined patients were treated with amniotic membrane graft due to the extreme severity of their ocular involvement, obtaining 3 years after treatment good visual acuity results and moderate or slight ocular damage sequels.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">When chronic involvement is established, sequels must be treated to avoid progression to blindness and ocular perforation. Corneal transplant has poor prognosis in these patients due to high rates of infection and perforation.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Accordingly, ophthalmologists play a crucial role in the evolution and ocular care of said diseases, establishing early treatment in the acute stage to avoid late sequels because, once these appear, it is difficult to restore the original anatomy and functionality of the structures.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Conflict of interests</span><p id="par0080" class="elsevierStylePara elsevierViewall">No conflict of interests was declared by the authors.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres833056" "titulo" => "Abstract" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Clinical cases" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Discussion" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec828978" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres833055" "titulo" => "Resumen" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "abst0020" "titulo" => "Introducción" ] 1 => array:2 [ "identificador" => "abst0025" "titulo" => "Casos clínicos" ] 2 => array:2 [ "identificador" => "abst0030" "titulo" => "Discusión" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec828979" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Clinic case reports" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflict of interests" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2016-05-09" "fechaAceptado" => "2016-10-13" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec828978" "palabras" => array:5 [ 0 => "Stevens–Johnson syndrome" 1 => "Toxic epidermal necrolysis" 2 => "Mycoplasma pneumonia" 3 => "Bullous mucocutaneous syndrome" 4 => "Mucous membranes" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec828979" "palabras" => array:5 [ 0 => "Síndrome Stevens Johnson" 1 => "Necrólisis epidérmica tóxica" 2 => "Mycoplasma pneumoniae" 3 => "Síndrome mucocutáneo ampolloso" 4 => "Membranas mucosas" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Stevens–Johnson syndrome and toxic epidermal necrolysis are two serious immune diseases within the context of bullous mucocutaneous syndrome. These have varying degrees of involvement of the skin and usually at least two mucous membranes.</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Clinical cases</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Three clinical cases are presented, two of them with significant ophthalmological sequelae, who had received drug treatment as a possible trigger, and another milder clinical case caused by Mycoplasma pneumoniae.</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Discussion</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The ophthalmologist plays a crucial role in the outcome and eye care of the patient in order to try to avoid the appearance of sequelae and subsequent loss of vision.</p></span>" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Clinical cases" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Discussion" ] ] ] "es" => array:3 [ "titulo" => "Resumen" "resumen" => "<span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Introducción</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Stevens-Johnson y la necrólisis epidérmica tóxica son 2 graves enfermedades inmunológicas dentro del contexto de síndrome mucocutáneo ampolloso, con diferente grado de afectación cutánea y que suelen presentar afectación de al menos 2 membranas mucosas.</p></span> <span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Casos clínicos</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Presentamos 3 casos clínicos, 2 de ellos con importantes secuelas oftalmológicas que habían recibido tratamiento farmacológico como posible desencadenante, y otro cuadro más larvado causado por Mycoplasma pneumoniae.</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Discusión</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">El oftalmólogo desempeña un papel crucial en la evolución y cuidados oculares del paciente para intentar evitar la aparición de secuelas y la consiguiente pérdida de visión.</p></span>" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "abst0020" "titulo" => "Introducción" ] 1 => array:2 [ "identificador" => "abst0025" "titulo" => "Casos clínicos" ] 2 => array:2 [ "identificador" => "abst0030" "titulo" => "Discusión" ] ] ] ] "NotaPie" => array:2 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Blanco N, Gutiérrez B, Valls I, Puertas D, Martín C, Rivera M, et al. Síndrome de Stevens-Johnson en la infancia. Arch Soc Esp Oftalmol. 2017;92:241–244.</p>" ] 1 => array:2 [ "etiqueta" => "☆☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">This paper was partially presented at the 24th Congress of the Strabology Society of Spain, April 14, 2016, Córdoba, Spain.</p>" ] ] "multimedia" => array:6 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 323 "Ancho" => 950 "Tamanyo" => 74523 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Ciliar injection (arrows) and pseudomembranous conjunctivitis in patient with atypical SJS after pneumonia due to Mycoplasma pneumoniae.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 769 "Ancho" => 950 "Tamanyo" => 132598 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Oral mucosa involvement in patient with atypical SJS after pneumonia due to Mycoplasma pneumoniae.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1130 "Ancho" => 750 "Tamanyo" => 128127 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Generalized exanthema with de-epithelized areas and Nyckolsky<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>(upper arrow), crusts (middle arrow) and bullous areas (lower arrow).</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Fig. 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 713 "Ancho" => 950 "Tamanyo" => 142600 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Corneal ulcer and formation of pseudomembranes in patient with TEN.</p>" ] ] 4 => array:7 [ "identificador" => "fig0025" "etiqueta" => "Fig. 5" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr5.jpeg" "Alto" => 998 "Ancho" => 950 "Tamanyo" => 105045 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Right eye severe ciliary injection, corneal ulcer with thinning and hypopyon level (arrow).</p>" ] ] 5 => array:7 [ "identificador" => "fig0030" "etiqueta" => "Fig. 6" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr6.jpeg" "Alto" => 741 "Ancho" => 900 "Tamanyo" => 101621 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Right eye with severe ciliary injection exhibiting corneal melting and herniation of spontaneous ocular content.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Stevens–Johnson syndrome/toxic epidermal necrolysys – a comprehensive review and guide to therapy. 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