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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Introducci&#243;n</span><p id="par0005" class="elsevierStylePara elsevierViewall">La pitiriasis rubra pilaris &#40;PRP&#41; es una enfermedad inflamatoria poco frecuente&#44; la cual se considera tradicionalmente como una dermatosis reactiva&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;3</span></a> Por edad se presentar&#225; con una distribuci&#243;n bimodal tanto en la primera como en la quinta d&#233;cadas de la vida&#44; y con una frecuencia similar entre ambos sexos&#46; Cl&#237;nicamente se caracterizar&#225; por p&#225;pulas y placas foliculares hiperquerat&#243;sicas de color salm&#243;n&#46; Algunos pacientes desarrollar&#225;n una eritrodermia con la presencia de islotes de piel no afectada&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">El diagn&#243;stico requiere de una buena correlaci&#243;n clinicopatol&#243;gica&#46; La principal caracter&#237;stica histopatol&#243;gica es la alternancia de ortoqueratosis y paraqueratosis&#44; conocida como &#8220;patr&#243;n en tablero de ajedrez&#8221;&#46; Las opciones de tratamiento incluyen diversos f&#225;rmacos&#44; como los corticosteroides t&#243;picos&#44; los retinoides orales&#44; los tratamientos inmunosupresores y los agentes biol&#243;gicos&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">2&#44;4&#8211;6</span></a> Se cree que la PRP comparte el mismo perfil de las citocinas presentes en la psoriasis&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">No existen directrices del tratamiento y las evidencias cient&#237;ficas actuales se limitan a informes y series de casos&#46; En consecuencia&#44; el tratamiento de la PRP representa un reto&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Objetivo</span><p id="par0020" class="elsevierStylePara elsevierViewall">Describir las caracter&#237;sticas clinicopatol&#243;gicas y demogr&#225;ficas de los pacientes con PRP y analizar las diversas modalidades de tratamiento&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">M&#233;todos</span><p id="par0025" class="elsevierStylePara elsevierViewall">Se realiz&#243; un estudio observacional retrospectivo multic&#233;ntrico en ocho hospitales espa&#241;oles donde se incluyeron pacientes con diagn&#243;stico clinicopatol&#243;gico de PRP&#44; tratados durante 20 a&#241;os &#40;entre enero de 2002 y febrero de 2022&#41;&#46; Las variables fueron recogidas por cada centro en una base de datos an&#243;nima&#46; En la muestra pedi&#225;trica se incluyeron aquellos pacientes menores de 16 a&#241;os&#46; Se utiliz&#243; el &#225;rea de superficie corporal &#40;ASC&#41; para evaluar la respuesta al tratamiento y se consider&#243; como respuesta completa al ASC &#60;3&#37;&#46; Se realiz&#243; un an&#225;lisis estad&#237;stico descriptivo&#44; utilizando la media y la desviaci&#243;n est&#225;ndar &#40;DE&#41; para las variables cuantitativas y frecuencias absolutas y relativas para las variables cualitativas&#46; Los contrastes de hip&#243;tesis en las variables cualitativas se realizaron mediante el test del Chi-cuadrado o el test exacto de Fisher y para las variables cuantitativas mediante el test de la T de Student&#46; Un valor p &#60;0&#44;05 se consider&#243; estad&#237;sticamente significativo&#46; Todos los an&#225;lisis se realizaron con el programa estad&#237;stico Stata&#174; &#40;StataCorp&#44; College Station&#44; Texas&#41; versi&#243;n 16 para Windows&#46; El estudio se realiz&#243; siguiendo la Declaraci&#243;n de Helsinki y fue aprobado por el Comit&#233; de &#201;tica M&#233;dica del Hospital 12 de Octubre &#40;n&#176;&#58; 22&#47;233&#41;&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Resultados</span><p id="par0030" class="elsevierStylePara elsevierViewall">Los resultados epidemiol&#243;gicos&#44; cl&#237;nicos&#44; histopatol&#243;gicos y terap&#233;uticos se pueden consultar en la <a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a>&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Epidemiolog&#237;a</span><p id="par0035" class="elsevierStylePara elsevierViewall">Se incluy&#243; a un total de 65 pacientes&#46; En el momento de la inclusi&#243;n&#44; ocho ni&#241;os ten&#237;an una edad media de 7&#44;8 a&#241;os y cuatro &#40;50&#37;&#41; eran mujeres&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Treinta y nueve pacientes varones representaban el 68&#37; de la muestra adulta y 40 de ellos &#40;70&#37;&#41; desarrollaron eritrodermia&#44; mientras que s&#243;lo un ni&#241;o la desarroll&#243;&#46; La edad media fue mayor en los adultos eritrod&#233;rmicos &#40;61 a&#241;os&#41; que en los no eritrod&#233;rmicos &#40;51 a&#241;os&#44; valor p<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>0&#44;015&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Factores desencadenantes y comorbilidades</span><p id="par0045" class="elsevierStylePara elsevierViewall">Trece pacientes &#40;20&#37;&#41; se&#241;alaron un posible desencadenante&#58; infecci&#243;n previa en siete &#40;12&#37;&#41; adultos y dos &#40;25&#37;&#41; ni&#241;os&#59; y f&#225;rmacos en cuatro &#40;7&#37;&#41; adultos&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">En los ni&#241;os&#44; todas las infecciones fueron de las v&#237;as respiratorias superiores&#46; Entre los pacientes adultos no recogimos datos espec&#237;ficos de agentes microbiol&#243;gicos&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Cuatro &#40;7&#37;&#41; de nuestros pacientes adultos ten&#237;an neoplasias concomitantes y cinco &#40;9&#37;&#41; comorbilidades autoinmunes&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Hallazgos cl&#237;nicos</span><p id="par0060" class="elsevierStylePara elsevierViewall">Las caracter&#237;sticas cl&#237;nicas m&#225;s frecuentes fueron la presencia de islotes de piel sana &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>49&#44; 83&#37;&#41; y la queratodermia palmoplantar &#40;PP&#41; &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>53&#44; 85&#37;&#41;&#44; que tambi&#233;n fueron los hallazgos cl&#237;nicos m&#225;s frecuentes en los pacientes eritrod&#233;rmicos &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">figs&#46; 1 y 2</a>&#41;&#46; La diferencia fue estad&#237;sticamente significativa en el caso de la queratodermia PP &#40;p-valor 0&#44;029&#41;&#46; Sin embargo&#44; los ni&#241;os s&#243;lo presentaron en dos &#40;29&#37;&#41; y cuatro &#40;50&#37;&#41; de los casos la presencia de islotes de piel sana y queratodermia PP&#44; respectivamente&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0065" class="elsevierStylePara elsevierViewall">Cuarenta y uno &#40;63&#37;&#41; de los pacientes refirieron prurito&#44; de los cuales s&#243;lo dos &#40;25&#37;&#41; eran ni&#241;os&#44; mientras que 39 &#40;68&#37;&#41; eran adultos &#40;p-valor 0&#44;017&#41;&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">Otras caracter&#237;sticas cl&#237;nicas se detallan en la <a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a>&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Histolog&#237;a</span><p id="par0075" class="elsevierStylePara elsevierViewall">Los hallazgos histol&#243;gicos m&#225;s frecuentes fueron la presencia de una alternancia entre la ortoqueratosis y la paraqueratosis &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>41&#44; 67&#37;&#41; y la hiperplasia epid&#233;rmica irregular &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>36&#44; 59&#37;&#41;&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Tratamiento</span><p id="par0080" class="elsevierStylePara elsevierViewall">La duraci&#243;n media del tratamiento fue de 2&#44;4 meses en ni&#241;os&#44; 8 meses en adultos no eritrod&#233;rmicos y 21&#44;4 meses en adultos eritrod&#233;rmicos&#46; El tiempo transcurrido hasta un BSA &#60;3&#37; fue m&#225;s corto y el n&#250;mero de tratamientos menor en los ni&#241;os y en los adultos no eritrod&#233;rmicos que en sus hom&#243;logos &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a>&#41;&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Todos los ni&#241;os fueron tratados inicialmente con corticosteroides t&#243;picos&#46; S&#243;lo uno de ellos precis&#243; acitretino&#46; Uno de los ni&#241;os desarroll&#243; una eritrodermia y necesit&#243; ustekinumab para controlar la enfermedad&#46; Se consigui&#243; una respuesta completa &#40;RC&#41; en siete &#40;88&#37;&#41; de los ni&#241;os&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">Los adultos no eritrod&#233;rmicos &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>17&#44; 30&#37;&#41; fueron tratados con &#233;xito con terapia t&#243;pica en nueve &#40;60&#37;&#41; pacientes&#46; Seis fueron tratados con agentes sist&#233;micos &#40;acitretino en tres y fototerapia en un paciente&#41;&#46; S&#243;lo cinco pacientes necesitaron un agente biol&#243;gico&#46; Se consigui&#243; una RC en 13 &#40;76&#37;&#41; de los adultos no eritrod&#233;rmicos&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Por otro lado&#44; 29 &#40;71&#37;&#41; pacientes eritrod&#233;rmicos fueron tratados inicialmente con agentes sist&#233;micos como acitretino en doce &#40;30&#37;&#41; de los casos o metotrexato en diez &#40;25&#37;&#41; de los pacientes&#44; fototerapia en siete &#40;18&#37;&#41; y un paciente comenz&#243; con ustekinumab&#46; El &#250;ltimo tratamiento que fue eficaz incluye una gran variedad de f&#225;rmacos &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a>&#41; y se consigui&#243; una respuesta completa en 26 &#40;68&#37;&#41; de los pacientes&#46; El uso de la terapia biol&#243;gica en alg&#250;n momento de la enfermedad fue necesaria en 31 &#40;68&#37;&#41; de los pacientes&#44; siendo el ustekinumab el tratamiento biol&#243;gico utilizado con mayor frecuencia&#46;</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Limitaciones</span><p id="par0100" class="elsevierStylePara elsevierViewall">Nuestro estudio tiene varias limitaciones&#46; En primer lugar&#44; nuestros datos se recogieron de forma retrospectiva con un tama&#241;o de muestra relativamente peque&#241;o&#46; Adem&#225;s&#44; no existen criterios diagn&#243;sticos estandarizados de PRP ni escalas de gravedad para evaluar la respuesta al tratamiento&#46; Por &#250;ltimo&#44; la disponibilidad de nuevas terapias biol&#243;gicas a lo largo de la &#250;ltima d&#233;cada puede haber ejercido cierta influencia en los resultados terap&#233;uticos de los pacientes diagnosticados en los &#250;ltimos a&#241;os&#46;</p></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Discusi&#243;n</span><p id="par0105" class="elsevierStylePara elsevierViewall">Este estudio presenta de manera detallada datos demogr&#225;ficos&#44; clinicopatol&#243;gicos y terap&#233;uticos de 65 pacientes diagnosticados de PRP&#46; Hasta donde sabemos&#44; se trata de la mayor serie europea de casos de pacientes con PRP&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">Nuestro estudio demostr&#243; predominio en pacientes adultos de sexo masculino &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>39&#44; 68&#37;&#41;&#44; lo que difiere de los datos de la literatura que mantienen una distribuci&#243;n equitativa por sexos&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;4</span></a> La distribuci&#243;n bimodal fue coherente con la literatura&#44;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;4</span></a> pero llamativamente los pacientes adultos que presentaban eritrodermia ten&#237;an una media de 10 a&#241;os m&#225;s &#40;valor p &#60;0&#44;05&#41;&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">En diversos informes de casos publicados se afirma que existe una posible causalidad entre neoplasias malignas o trastornos autoinmunitarios y la PRP&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;7</span></a> Sin embargo&#44; nuestros pacientes adultos mostraron una baja frecuencia de comorbilidades autoinmunitarias &#40;9&#37;&#41; y neoplasias malignas &#40;6&#37;&#41;&#44; lo que sugiere que su frecuencia puede no diferir de la poblaci&#243;n general y que los procedimientos de cribado deben guiarse por la historia cl&#237;nica y la exploraci&#243;n f&#237;sica&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">Habitualmente se han descrito agentes infecciosos y farmacol&#243;gicos como desencadenantes de la PRP&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;2</span></a> Curiosamente&#44; el 25&#37; de los ni&#241;os de nuestro estudio refirieron el antecedente de un desencadenante infeccioso&#44; pero s&#243;lo en el 6&#37; de los adultos eritrod&#233;rmicos se demostr&#243; la presencia de un f&#225;rmaco que fuese responsable&#46; Esto apoya la idea de que la PRP es una enfermedad multifactorial&#44; en la que los factores ambientales desempe&#241;an un papel&#44; pero no son necesarios para el desarrollo de la enfermedad&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">La caracter&#237;stica histol&#243;gica m&#225;s frecuente fue la alternancia entre la ortoqueratosis y la paraqueratosis&#44; lo que coincide con los datos presentados en la literatura&#46; Sin embargo&#44; la histolog&#237;a puede ser inespec&#237;fica y a menudo el diagn&#243;stico se gu&#237;a predominantemente por los rasgos cl&#237;nicos caracter&#237;sticos&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;4</span></a> El 66&#37; de nuestros pacientes presentaron inicialmente lesiones en la parte superior del cuerpo&#44; lo que concuerda con la progresi&#243;n cefalocaudal descrita cl&#225;sicamente&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;4</span></a> Curiosamente&#44; el 94&#37; de nuestros pacientes eritrod&#233;rmicos presentaron queratodermia PP &#40;valor p 0&#44;02&#41;&#46;</p><p id="par0130" class="elsevierStylePara elsevierViewall">Fouarge et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> ya propusieron la queratodermia PP como posible factor de resistencia a un antagonista de IL-23&#47;p19&#46; En nuestra opini&#243;n&#44; puede ser un marcador de progresi&#243;n y podr&#237;a ayudar a identificar a los pacientes con mayor riesgo de desarrollar formas eritrod&#233;rmicas de PRP&#46;</p><p id="par0135" class="elsevierStylePara elsevierViewall">El 75&#37; de los pacientes pedi&#225;tricos y el 60&#37; de los adultos no eritrod&#233;rmicos controlaron su enfermedad con corticosteroides t&#243;picos&#46; La buena respuesta a la terapia t&#243;pica ya se describi&#243; en la PRP pedi&#225;trica&#44;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a> pero suponemos que es extensible a los adultos con enfermedad localizada&#46; Sin embargo&#44; la mayor&#237;a de los pacientes eritrod&#233;rmicos &#40;68&#37;&#41; requirieron terapia biol&#243;gica como &#250;ltima l&#237;nea terap&#233;utica eficaz&#46; Entre ellos&#44; el 68&#37; alcanz&#243; respuesta completa&#44; pero tras una media de 6&#44;5 meses de tratamiento&#46; Adem&#225;s&#44; estos suelen recibir m&#225;s l&#237;neas de tratamiento &#40;media de 2&#44;7&#41; que los pacientes con lesiones localizadas y pueden beneficiarse de un abordaje m&#225;s invasivo y precoz&#46;</p></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Conclusi&#243;n</span><p id="par0140" class="elsevierStylePara elsevierViewall">En conclusi&#243;n&#44; en nuestro estudio se evidenci&#243; que la PRP puede ser m&#225;s frecuente en hombres y que las formas eritrod&#233;rmicas podr&#237;an aparecer en pacientes de edad avanzada&#46; No hubo una asociaci&#243;n clara con el c&#225;ncer o las enfermedades autoinmunes en nuestros pacientes&#46; La queratodermia PP puede ser un marcador de progresi&#243;n a la eritrodermia&#46; En nuestra muestra los ni&#241;os y los adultos que presentaban una enfermedad localizada respondieron a los corticosteroides t&#243;picos&#46; Por el contrario&#44; los pacientes eritrod&#233;rmicos recibieron m&#250;ltiples l&#237;neas terap&#233;uticas&#44; adem&#225;s de necesitar una media de tratamiento de 6&#44;5 meses y un agente biol&#243;gico para poder controlar su enfermedad&#46; Se necesitan m&#225;s estudios para investigar esta dif&#237;cil enfermedad&#46;</p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0140">Consentimiento Informado de los pacientes</span><p id="par0145" class="elsevierStylePara elsevierViewall">Los pacientes de este manuscrito han dado su consentimiento informado por escrito para la publicaci&#243;n de los detalles de sus casos&#46;</p></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0145">Financiaci&#243;n</span><p id="par0150" class="elsevierStylePara elsevierViewall">No se declara ninguna&#46;</p></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0150">Conflictos de intereses</span><p id="par0155" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">-</span><p id="par0160" class="elsevierStylePara elsevierViewall">El Dr&#46; Ruiz Genao ha sido remunerado por Almirall&#44; Pfizer&#44; Janssen&#44; Amgen&#44; Abbvie&#44; Lilly&#44; UCB&#44; Novartis y Leo-Pharma por servicios de asesoramiento y conferencias&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">-</span><p id="par0165" class="elsevierStylePara elsevierViewall">L&#46; Puig ha recibido honorarios como consultor&#47;ponente y&#47;o ha participado en ensayos cl&#237;nicos patrocinados por Abbvie&#44; Almirall&#44; Amgen&#44; Biogen&#44; Boehringer Ingelheim&#44; Bristol Myers Squibb&#44; Janssen&#44; Leo-Pharma&#44; Lilly&#44; Novartis&#44; Pfizer&#44; Sandoz&#44; Sanofi y UCB&#46;</p></li></ul></p></span></span>"
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    "fechaRecibido" => "2023-09-24"
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            0 => "Pitiriasis <span class="elsevierStyleItalic">rubra pilaris</span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Introducci&#243;n</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La pitiriasis <span class="elsevierStyleItalic">rubra pilaris</span> &#40;PRP&#41; es una entidad rara de etiopatogenia desconocida&#46; La falta de gu&#237;as cl&#237;nicas la convierte en un desaf&#237;o para los cl&#237;nicos&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Objetivo</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Contribuir con nuestra experiencia a aumentar la evidencia disponible sobre esta entidad&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">M&#233;todo</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Realizamos un estudio retrospectivo&#44; descriptivo y multic&#233;ntrico de 65 pacientes con PRP&#44; siendo la serie de casos europea m&#225;s grande de pacientes con PRP&#46;</p></span> <span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Resultados</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">La PRP fue m&#225;s frecuente en los varones con una edad promedio de 51 a&#241;os&#44; pero las formas eritrod&#233;rmicas se presentaron en pacientes de mayor edad&#44; en torno a los 61 a&#241;os&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Seis &#40;75&#37;&#41; de los pacientes pedi&#225;tricos y 10 &#40;60&#37;&#41; de los adultos no eritrod&#233;rmicos controlaron su enfermedad con corticoides t&#243;picos&#46; Por el contrario&#44; 26 &#40;68&#37;&#41; de los pacientes eritrod&#233;rmicos necesitaron terapia biol&#243;gica como &#250;ltima l&#237;nea terap&#233;utica eficaz&#59; requiriendo un promedio de 6&#44;5 meses para lograr una respuesta completa&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conclusi&#243;n</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Nuestro estudio mostr&#243; una diferencia estad&#237;stica en t&#233;rminos de resultado y respuesta al tratamiento entre ni&#241;os o pacientes con enfermedad limitada y pacientes que desarrollan eritrodermia&#46;</p></span>"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Introduction</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">PRP is a rare entity of unknown etiopathogenesis&#46; Lack of management guidelines makes it a challenge for clinicians&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Objective</span><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">To add our experience to increase evidence about PRP&#46;</p></span> <span id="abst0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Methods</span><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">We performed a retrospective&#44; descriptive and multicentric study of 65 patients with PRP&#44; being the largest European case series of patients with PRP&#46;</p></span> <span id="abst0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Results</span><p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">PRP was more frequent in male patients with an average age of 51 years&#44; but erythrodermic forms presented in older patients &#40;average age 61 years&#41;&#46;</p><p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Six &#40;75&#37;&#41; paediatric patients and ten &#40;60&#37;&#41; non-erythrodermic adults controlled their disease with topical corticosteroids&#46; On the contrary&#44; 26 &#40;68&#37;&#41; erythrodermic patients required biologic therapy as last and effective therapy line requiring an average of 6&#46;5 months to achieve complete response&#46;</p></span> <span id="abst0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conclusion</span><p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Our study showed a statistical difference in terms of outcome and response to treatment between children or patients with limited disease and patients who develop erythroderma&#46;</p></span>"
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Droga&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Comorbilidades</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Autoinmunes&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Neoplasias&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Hallazgos cl&#237;nicos</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Lesiones iniciales en la parte superior del cuerpo&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Prurito&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">39 &#40;68&#37;&#41;<a class="elsevierStyleCrossRef" href="#tblfn0015">&#42;</a>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">9 &#40;53&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Afectaci&#243;n ungueal&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Eritrodermia&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">1 &#40;13&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">40 &#40;70&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">100&#37;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Islotes de piel sana no comprometidas&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">49 &#40;83&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2 &#40;29&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">47 &#40;90&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">10 &#40;77&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">36 &#40;95&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a>Queratodermia PP&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">53 &#40;82&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">4 &#40;50&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">49 &#40;86&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">12 &#40;71&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">37 &#40;93&#37;&#41;<a class="elsevierStyleCrossRef" href="#tblfn0015">&#42;</a>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Afectaci&#243;n intertriginosa&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Hallazgos histopatol&#243;gicos</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Alternancia de ortoqueratosis y paraqueratosis&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Hiperplasia irregular de la epidermis&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Paraqueratosis peri-infundibular&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Acitretino&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Corticosteroides t&#243;picos&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Tratamientos efectivos de &#250;ltima l&#237;nea</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#40;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Infliximab&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 &#40;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 &#40;7&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 &#40;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Ixekizumab&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 &#40;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 &#40;7&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0 &#40;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">4 &#40;10&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Risankizumab&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0 &#40;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;3&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Secukinumab&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 &#40;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 &#40;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;6&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 &#40;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Ustekinumab&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">15 &#40;23&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1 &#40;13&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">14 &#40;25&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2 &#40;12&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">12 &#40;31&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Brodalumab&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 &#40;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 &#40;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0 &#40;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3 &#40;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="7" align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Respuesta completa</span><a class="elsevierStyleCrossRef" href="#tblfn0020">&#42;&#42;</a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">46 &#40;73&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7 &#40;88&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">39 &#40;71&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">13 &#40;76&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">26 &#40;68&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Tiempo hasta el BSA &#60;3&#37; &#40;meses&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">5 &#40;3&#44;4&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#44;4 &#40;0&#44;8&#41;<a class="elsevierStyleCrossRef" href="#tblfn0015">&#42;</a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">5&#44;4 &#40;3&#44;5&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3&#44;2 &#40;2&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6&#44;5 &#40;3&#44;5&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Duraci&#243;n del tratamiento</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">16&#44;5 &#40;23&#44;9&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#44;4 &#40;0&#44;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">18&#44;1 &#40;24&#44;8&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">8&#44;0 &#40;6&#44;9&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">21&#44;5 &#40;27&#44;6&#41;&#42;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
              "imagenFichero" => array:1 [
                0 => "xTab3637925.png"
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            0 => array:3 [
              "identificador" => "tblfn0005"
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              "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Queratodermia PP&#58; queratodermia palmoplantar&#46;</p>"
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Original
Pitiriasis rubra pilaris: serie de casos multicéntrica de 65 pacientes españoles
Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients
J. Montero-Menárgueza,
Corresponding author
julia.montero.menarguez@gmail.com

Autor para correspondencia.
, V. Amat Samaranchb, L. Puig Sanzb, R. Ruiz-Villaverdec, S. Arias-Santiagod, M. Larrea Garcíae, D. Ruiz Genaof, M. Ferráng, L. Schneller-Pavelescuh, C. Romero Ferreiroi,j, R. Rivera Díaza
a Dermatology Department of the Hospital Universitario 12 de Octubre, Madrid, España
b Dermatology Department of Hospital de la Santa Creu i Sant Pau de Barcelona, España
c Dermatology Department of Hospital Universitario San Cecilio de Granada, España
d Dermatology Department of Hospital Virgen de las Nieves de Granada, School of Medicine, Granada University, Instituto de Investigación Biosanitaria IBS, Granada, España
e Dermatology Department of Hospital Universitario de Navarra, España
f Dermatology Department of Fundación Hospital Alcorcón, España
g Dermatology Department of Hospital del Mar de Barcelona, España
h Dermatology Department of Hospital de la Vega Baja de Alicante, España
i Unidad de Soporte Científico, Instituto de Investigación Sanitaria, Hospital Universitario 12 de Octubre, España
j Facultad de Ciencias de la Salud, Universidad Francisco de Vitoria, Pozuelo de Alarcón, Madrid, España
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La principal caracter&#237;stica histopatol&#243;gica es la alternancia de ortoqueratosis y paraqueratosis&#44; conocida como &#8220;patr&#243;n en tablero de ajedrez&#8221;&#46; Las opciones de tratamiento incluyen diversos f&#225;rmacos&#44; como los corticosteroides t&#243;picos&#44; los retinoides orales&#44; los tratamientos inmunosupresores y los agentes biol&#243;gicos&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">2&#44;4&#8211;6</span></a> Se cree que la PRP comparte el mismo perfil de las citocinas presentes en la psoriasis&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">No existen directrices del tratamiento y las evidencias cient&#237;ficas actuales se limitan a informes y series de casos&#46; En consecuencia&#44; el tratamiento de la PRP representa un reto&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Objetivo</span><p id="par0020" class="elsevierStylePara elsevierViewall">Describir las caracter&#237;sticas clinicopatol&#243;gicas y demogr&#225;ficas de los pacientes con PRP y analizar las diversas modalidades de tratamiento&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">M&#233;todos</span><p id="par0025" class="elsevierStylePara elsevierViewall">Se realiz&#243; 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a un total de 65 pacientes&#46; En el momento de la inclusi&#243;n&#44; ocho ni&#241;os ten&#237;an una edad media de 7&#44;8 a&#241;os y cuatro &#40;50&#37;&#41; eran mujeres&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Treinta y nueve pacientes varones representaban el 68&#37; de la muestra adulta y 40 de ellos &#40;70&#37;&#41; desarrollaron eritrodermia&#44; mientras que s&#243;lo un ni&#241;o la desarroll&#243;&#46; La edad media fue mayor en los adultos eritrod&#233;rmicos &#40;61 a&#241;os&#41; que en los no eritrod&#233;rmicos &#40;51 a&#241;os&#44; valor p<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>0&#44;015&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Factores desencadenantes y comorbilidades</span><p id="par0045" class="elsevierStylePara elsevierViewall">Trece pacientes &#40;20&#37;&#41; se&#241;alaron un posible desencadenante&#58; infecci&#243;n previa en siete &#40;12&#37;&#41; adultos y dos &#40;25&#37;&#41; ni&#241;os&#59; y f&#225;rmacos en cuatro &#40;7&#37;&#41; adultos&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">En los ni&#241;os&#44; todas las infecciones fueron de las v&#237;as respiratorias superiores&#46; Entre los pacientes adultos no recogimos datos espec&#237;ficos de agentes microbiol&#243;gicos&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Cuatro &#40;7&#37;&#41; de nuestros pacientes adultos ten&#237;an neoplasias concomitantes y cinco &#40;9&#37;&#41; comorbilidades autoinmunes&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Hallazgos cl&#237;nicos</span><p id="par0060" class="elsevierStylePara elsevierViewall">Las caracter&#237;sticas cl&#237;nicas m&#225;s frecuentes fueron la presencia de islotes de piel sana &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>49&#44; 83&#37;&#41; y la queratodermia palmoplantar &#40;PP&#41; &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>53&#44; 85&#37;&#41;&#44; que tambi&#233;n fueron los hallazgos cl&#237;nicos m&#225;s frecuentes en los pacientes eritrod&#233;rmicos &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">figs&#46; 1 y 2</a>&#41;&#46; La diferencia fue estad&#237;sticamente significativa en el caso de la queratodermia PP &#40;p-valor 0&#44;029&#41;&#46; Sin embargo&#44; los ni&#241;os s&#243;lo presentaron en dos &#40;29&#37;&#41; y cuatro &#40;50&#37;&#41; de los casos la presencia de islotes de piel sana y queratodermia PP&#44; respectivamente&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0065" class="elsevierStylePara elsevierViewall">Cuarenta y uno &#40;63&#37;&#41; de los pacientes refirieron prurito&#44; de los cuales s&#243;lo dos &#40;25&#37;&#41; eran ni&#241;os&#44; mientras que 39 &#40;68&#37;&#41; eran adultos &#40;p-valor 0&#44;017&#41;&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">Otras caracter&#237;sticas cl&#237;nicas se detallan en la <a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a>&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Histolog&#237;a</span><p id="par0075" class="elsevierStylePara elsevierViewall">Los hallazgos histol&#243;gicos m&#225;s frecuentes fueron la presencia de una alternancia entre la ortoqueratosis y la paraqueratosis &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>41&#44; 67&#37;&#41; y la hiperplasia epid&#233;rmica irregular &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>36&#44; 59&#37;&#41;&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Tratamiento</span><p id="par0080" class="elsevierStylePara elsevierViewall">La duraci&#243;n media del tratamiento fue de 2&#44;4 meses en ni&#241;os&#44; 8 meses en adultos no eritrod&#233;rmicos y 21&#44;4 meses en adultos eritrod&#233;rmicos&#46; El tiempo transcurrido hasta un BSA &#60;3&#37; fue m&#225;s corto y el n&#250;mero de tratamientos menor en los ni&#241;os y en los adultos no eritrod&#233;rmicos que en sus hom&#243;logos &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a>&#41;&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Todos los ni&#241;os fueron tratados inicialmente con corticosteroides t&#243;picos&#46; S&#243;lo uno de ellos precis&#243; acitretino&#46; Uno de los ni&#241;os desarroll&#243; una eritrodermia y necesit&#243; ustekinumab para controlar la enfermedad&#46; Se consigui&#243; una respuesta completa &#40;RC&#41; en siete &#40;88&#37;&#41; de los ni&#241;os&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">Los adultos no eritrod&#233;rmicos &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>17&#44; 30&#37;&#41; fueron tratados con &#233;xito con terapia t&#243;pica en nueve &#40;60&#37;&#41; pacientes&#46; Seis fueron tratados con agentes sist&#233;micos &#40;acitretino en tres y fototerapia en un paciente&#41;&#46; S&#243;lo cinco pacientes necesitaron un agente biol&#243;gico&#46; Se consigui&#243; una RC en 13 &#40;76&#37;&#41; de los adultos no eritrod&#233;rmicos&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Por otro lado&#44; 29 &#40;71&#37;&#41; pacientes eritrod&#233;rmicos fueron tratados inicialmente con agentes sist&#233;micos como acitretino en doce &#40;30&#37;&#41; de los casos o metotrexato en diez &#40;25&#37;&#41; de los pacientes&#44; fototerapia en siete &#40;18&#37;&#41; y un paciente comenz&#243; con ustekinumab&#46; El &#250;ltimo tratamiento que fue eficaz incluye una gran variedad de f&#225;rmacos &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a>&#41; y se consigui&#243; una respuesta completa en 26 &#40;68&#37;&#41; de los pacientes&#46; El uso de la terapia biol&#243;gica en alg&#250;n momento de la enfermedad fue necesaria en 31 &#40;68&#37;&#41; de los pacientes&#44; siendo el ustekinumab el tratamiento biol&#243;gico utilizado con mayor frecuencia&#46;</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Limitaciones</span><p id="par0100" class="elsevierStylePara elsevierViewall">Nuestro estudio tiene varias limitaciones&#46; En primer lugar&#44; nuestros datos se recogieron de forma retrospectiva con un tama&#241;o de muestra relativamente peque&#241;o&#46; Adem&#225;s&#44; no existen criterios diagn&#243;sticos estandarizados de PRP ni escalas de gravedad para evaluar la respuesta al tratamiento&#46; Por &#250;ltimo&#44; la disponibilidad de nuevas terapias biol&#243;gicas a lo largo de la &#250;ltima d&#233;cada puede haber ejercido cierta influencia en los resultados terap&#233;uticos de los pacientes diagnosticados en los &#250;ltimos a&#241;os&#46;</p></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Discusi&#243;n</span><p id="par0105" class="elsevierStylePara elsevierViewall">Este estudio presenta de manera detallada datos demogr&#225;ficos&#44; clinicopatol&#243;gicos y terap&#233;uticos de 65 pacientes diagnosticados de PRP&#46; Hasta donde sabemos&#44; se trata de la mayor serie europea de casos de pacientes con PRP&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">Nuestro estudio demostr&#243; predominio en pacientes adultos de sexo masculino &#40;n<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>39&#44; 68&#37;&#41;&#44; lo que difiere de los datos de la literatura que mantienen una distribuci&#243;n equitativa por sexos&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;4</span></a> La distribuci&#243;n bimodal fue coherente con la literatura&#44;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;4</span></a> pero llamativamente los pacientes adultos que presentaban eritrodermia ten&#237;an una media de 10 a&#241;os m&#225;s &#40;valor p &#60;0&#44;05&#41;&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">En diversos informes de casos publicados se afirma que existe una posible causalidad entre neoplasias malignas o trastornos autoinmunitarios y la PRP&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;7</span></a> Sin embargo&#44; nuestros pacientes adultos mostraron una baja frecuencia de comorbilidades autoinmunitarias &#40;9&#37;&#41; y neoplasias malignas &#40;6&#37;&#41;&#44; lo que sugiere que su frecuencia puede no diferir de la poblaci&#243;n general y que los procedimientos de cribado deben guiarse por la historia cl&#237;nica y la exploraci&#243;n f&#237;sica&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">Habitualmente se han descrito agentes infecciosos y farmacol&#243;gicos como desencadenantes de la PRP&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;2</span></a> Curiosamente&#44; el 25&#37; de los ni&#241;os de nuestro estudio refirieron el antecedente de un desencadenante infeccioso&#44; pero s&#243;lo en el 6&#37; de los adultos eritrod&#233;rmicos se demostr&#243; la presencia de un f&#225;rmaco que fuese responsable&#46; Esto apoya la idea de que la PRP es una enfermedad multifactorial&#44; en la que los factores ambientales desempe&#241;an un papel&#44; pero no son necesarios para el desarrollo de la enfermedad&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">La caracter&#237;stica histol&#243;gica m&#225;s frecuente fue la alternancia entre la ortoqueratosis y la paraqueratosis&#44; lo que coincide con los datos presentados en la literatura&#46; Sin embargo&#44; la histolog&#237;a puede ser inespec&#237;fica y a menudo el diagn&#243;stico se gu&#237;a predominantemente por los rasgos cl&#237;nicos caracter&#237;sticos&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;4</span></a> El 66&#37; de nuestros pacientes presentaron inicialmente lesiones en la parte superior del cuerpo&#44; lo que concuerda con la progresi&#243;n cefalocaudal descrita cl&#225;sicamente&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;4</span></a> Curiosamente&#44; el 94&#37; de nuestros pacientes eritrod&#233;rmicos presentaron queratodermia PP &#40;valor p 0&#44;02&#41;&#46;</p><p id="par0130" class="elsevierStylePara elsevierViewall">Fouarge et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> ya propusieron la queratodermia PP como posible factor de resistencia a un antagonista de IL-23&#47;p19&#46; En nuestra opini&#243;n&#44; puede ser un marcador de progresi&#243;n y podr&#237;a ayudar a identificar a los pacientes con mayor riesgo de desarrollar formas eritrod&#233;rmicas de PRP&#46;</p><p id="par0135" class="elsevierStylePara elsevierViewall">El 75&#37; de los pacientes pedi&#225;tricos y el 60&#37; de los adultos no eritrod&#233;rmicos controlaron su enfermedad con corticosteroides t&#243;picos&#46; La buena respuesta a la terapia t&#243;pica ya se describi&#243; en la PRP pedi&#225;trica&#44;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a> pero suponemos que es extensible a los adultos con enfermedad localizada&#46; Sin embargo&#44; la mayor&#237;a de los pacientes eritrod&#233;rmicos &#40;68&#37;&#41; requirieron terapia biol&#243;gica como &#250;ltima l&#237;nea terap&#233;utica eficaz&#46; Entre ellos&#44; el 68&#37; alcanz&#243; respuesta completa&#44; pero tras una media de 6&#44;5 meses de tratamiento&#46; Adem&#225;s&#44; estos suelen recibir m&#225;s l&#237;neas de tratamiento &#40;media de 2&#44;7&#41; que los pacientes con lesiones localizadas y pueden beneficiarse de un abordaje m&#225;s invasivo y precoz&#46;</p></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Conclusi&#243;n</span><p id="par0140" class="elsevierStylePara elsevierViewall">En conclusi&#243;n&#44; en nuestro estudio se evidenci&#243; que la PRP puede ser m&#225;s frecuente en hombres y que las formas eritrod&#233;rmicas podr&#237;an aparecer en pacientes de edad avanzada&#46; No hubo una asociaci&#243;n clara con el c&#225;ncer o las enfermedades autoinmunes en nuestros pacientes&#46; La queratodermia PP puede ser un marcador de progresi&#243;n a la eritrodermia&#46; En nuestra muestra los ni&#241;os y los adultos que presentaban una enfermedad localizada respondieron a los corticosteroides t&#243;picos&#46; Por el contrario&#44; los pacientes eritrod&#233;rmicos recibieron m&#250;ltiples l&#237;neas terap&#233;uticas&#44; adem&#225;s de necesitar una media de tratamiento de 6&#44;5 meses y un agente biol&#243;gico para poder controlar su enfermedad&#46; Se necesitan m&#225;s estudios para investigar esta dif&#237;cil enfermedad&#46;</p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0140">Consentimiento Informado de los pacientes</span><p id="par0145" class="elsevierStylePara elsevierViewall">Los pacientes de este manuscrito han dado su consentimiento informado por escrito para la publicaci&#243;n de los detalles de sus casos&#46;</p></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0145">Financiaci&#243;n</span><p id="par0150" class="elsevierStylePara elsevierViewall">No se declara ninguna&#46;</p></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0150">Conflictos de intereses</span><p id="par0155" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">-</span><p id="par0160" class="elsevierStylePara elsevierViewall">El Dr&#46; Ruiz Genao ha sido remunerado por Almirall&#44; Pfizer&#44; Janssen&#44; Amgen&#44; Abbvie&#44; Lilly&#44; UCB&#44; Novartis y Leo-Pharma por servicios de asesoramiento y conferencias&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">-</span><p id="par0165" class="elsevierStylePara elsevierViewall">L&#46; Puig ha recibido honorarios como consultor&#47;ponente y&#47;o ha participado en ensayos cl&#237;nicos patrocinados por Abbvie&#44; Almirall&#44; Amgen&#44; Biogen&#44; Boehringer Ingelheim&#44; Bristol Myers Squibb&#44; Janssen&#44; Leo-Pharma&#44; Lilly&#44; Novartis&#44; Pfizer&#44; Sandoz&#44; Sanofi y UCB&#46;</p></li></ul></p></span></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Introducci&#243;n</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La pitiriasis <span class="elsevierStyleItalic">rubra pilaris</span> &#40;PRP&#41; es una entidad rara de etiopatogenia desconocida&#46; La falta de gu&#237;as cl&#237;nicas la convierte en un desaf&#237;o para los cl&#237;nicos&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Objetivo</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Contribuir con nuestra experiencia a aumentar la evidencia disponible sobre esta entidad&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">M&#233;todo</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Realizamos un estudio retrospectivo&#44; descriptivo y multic&#233;ntrico de 65 pacientes con PRP&#44; siendo la serie de casos europea m&#225;s grande de pacientes con PRP&#46;</p></span> <span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Resultados</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">La PRP fue m&#225;s frecuente en los varones con una edad promedio de 51 a&#241;os&#44; pero las formas eritrod&#233;rmicas se presentaron en pacientes de mayor edad&#44; en torno a los 61 a&#241;os&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Seis &#40;75&#37;&#41; de los pacientes pedi&#225;tricos y 10 &#40;60&#37;&#41; de los adultos no eritrod&#233;rmicos controlaron su enfermedad con corticoides t&#243;picos&#46; Por el contrario&#44; 26 &#40;68&#37;&#41; de los pacientes eritrod&#233;rmicos necesitaron terapia biol&#243;gica como &#250;ltima l&#237;nea terap&#233;utica eficaz&#59; requiriendo un promedio de 6&#44;5 meses para lograr una respuesta completa&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conclusi&#243;n</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Nuestro estudio mostr&#243; una diferencia estad&#237;stica en t&#233;rminos de resultado y respuesta al tratamiento entre ni&#241;os o pacientes con enfermedad limitada y pacientes que desarrollan eritrodermia&#46;</p></span>"
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        "resumen" => "<span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Introduction</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">PRP is a rare entity of unknown etiopathogenesis&#46; Lack of management guidelines makes it a challenge for clinicians&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Objective</span><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">To add our experience to increase evidence about PRP&#46;</p></span> <span id="abst0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Methods</span><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">We performed a retrospective&#44; descriptive and multicentric study of 65 patients with PRP&#44; being the largest European case series of patients with PRP&#46;</p></span> <span id="abst0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Results</span><p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">PRP was more frequent in male patients with an average age of 51 years&#44; but erythrodermic forms presented in older patients &#40;average age 61 years&#41;&#46;</p><p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Six &#40;75&#37;&#41; paediatric patients and ten &#40;60&#37;&#41; non-erythrodermic adults controlled their disease with topical corticosteroids&#46; On the contrary&#44; 26 &#40;68&#37;&#41; erythrodermic patients required biologic therapy as last and effective therapy line requiring an average of 6&#46;5 months to achieve complete response&#46;</p></span> <span id="abst0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conclusion</span><p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Our study showed a statistical difference in terms of outcome and response to treatment between children or patients with limited disease and patients who develop erythroderma&#46;</p></span>"
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Neoplasias&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " colspan="7" align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Hallazgos cl&#237;nicos</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Lesiones iniciales en la parte superior del cuerpo&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Prurito&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">39 &#40;68&#37;&#41;<a class="elsevierStyleCrossRef" href="#tblfn0015">&#42;</a>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Afectaci&#243;n ungueal&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Eritrodermia&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Islotes de piel sana no comprometidas&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">10 &#40;77&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a>Queratodermia PP&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">49 &#40;86&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">12 &#40;71&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">37 &#40;93&#37;&#41;<a class="elsevierStyleCrossRef" href="#tblfn0015">&#42;</a>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Afectaci&#243;n intertriginosa&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">20 &#40;31&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">18 &#40;32&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">7 &#40;41&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">11 &#40;28&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t  " colspan="7" align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Hallazgos histopatol&#243;gicos</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Alternancia de ortoqueratosis y paraqueratosis&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">41 &#40;67&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">3 &#40;60&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">38 &#40;68&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">11 &#40;65&#37;&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Hiperplasia irregular de la epidermis&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Paraqueratosis peri-infundibular&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">N&#176; de biopsias</span>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">N&#176; de tratamientos</span>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">0&#44;8 &#40;0&#44;7&#41;<a class="elsevierStyleCrossRef" href="#tblfn0015">&#42;</a>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Tratamientos de primera l&#237;nea</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Acitretino&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Ciclosporina&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Corticosteroides t&#243;picos&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Metotrexate&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>UVB-BE<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Corticosteroides t&#243;picos&nbsp;\t\t\t\t\t\t\n
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                          "autores" => array:4 [
                            0 => "D&#46; Wang"
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                        "volumen" => "32"
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Article information
ISSN: 00017310
Original language: Spanish
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