{"id":10844,"date":"2020-11-18T03:41:29","date_gmt":"2020-11-18T03:41:29","guid":{"rendered":"https:\/\/dermatly.com\/pseudoxantoma-elastico\/"},"modified":"2026-03-10T09:55:48","modified_gmt":"2026-03-10T09:55:48","slug":"pseudoxantoma-elastico","status":"publish","type":"glossary","link":"https:\/\/dermatly.com\/it\/pseudoxantoma-elastico\/","title":{"rendered":"Pseudoxantoma elastico"},"content":{"rendered":"<section class=\"textBlock\">\n<h2>\u00bfQu\u00e9 es el pseudoxantoma el\u00e1stico?<\/h2>\n<p>Pseudoxantoma el\u00e1stico (PXE) es el nombre que se le da a un grupo de <span class=\"term\" data-term-id=\"43\">tejido conectivo<\/span> trastornos que afectan el tejido el\u00e1stico de la piel, <span class=\"term\" data-term-id=\"953\">vasos sanguineos<\/span>y los ojos.  PXE tambi\u00e9n se conoce como Gronblad-Strandberg <span class=\"term\" data-term-id=\"350\">s\u00edndrome<\/span>.<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\">Pseudoxantoma el\u00e1stico<\/h5>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img decoding=\"async\" src=\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/pxe__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd.jpg\" alt=\"PXE\"><\/div>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img decoding=\"async\" src=\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/pxe-antecubital3__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsInkiLDI4XQ.jpg\" alt=\"Pseudoxantoma el\u00e1stico de la fosa antecubital\"><\/div>\n<p class=\"p-small\">Pseudoxantoma el\u00e1stico de la fosa antecubital<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img decoding=\"async\" src=\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/pxe2__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsInkiLDM2XQ.jpg\" alt=\"PXE\"><\/div>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<h2>\u00bfCu\u00e1l es la causa del pseudoxantoma el\u00e1stico?<\/h2>\n<p>La falla b\u00e1sica en PXE parece estar relacionada con <span class=\"term\" data-term-id=\"558\">gen\u00e9tico<\/span> <span class=\"term\" data-term-id=\"1935\">anormalidades<\/span> en <span class=\"term\" data-term-id=\"1271\">cromosoma<\/span> 16p13.1.  La enfermedad involucra al transportador <span class=\"term\" data-term-id=\"775\">genes<\/span> MRP6 o ABCC6 en al menos algunas familias afectadas.  Hay buena evidencia de que <span class=\"term\" data-term-id=\"39\">col\u00e1geno<\/span> y <span class=\"term\" data-term-id=\"115\">sustancia del suelo<\/span> son anormales en las personas afectadas.  El calcio luego se acumula en forma anormal <span class=\"term\" data-term-id=\"1615\">fibras el\u00e1sticas<\/span> en la piel, vasos sangu\u00edneos, ojos y coraz\u00f3n.<\/p>\n<p>PXE afecta aproximadamente a 1 de cada 160.000 personas.  Hay varios subtipos de PXE, cada uno con una presentaci\u00f3n cl\u00ednica y un patr\u00f3n de herencia ligeramente diferentes.  Dos se heredan como <span class=\"term\" data-term-id=\"828\">autos\u00f3mico<\/span> caracter\u00edstica dominante;  esto significa que la mitad de los hijos de una persona afectada se ven afectados.  Tres tipos de PXE son <span class=\"term\" data-term-id=\"830\">autos\u00f3mica recesiva<\/span>, en el que 1 de cada 4 ni\u00f1os se ven afectados.<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n                        <!-- \/340621630\/dermnet_mobile_afterfirstsection_300x250 --><\/p>\n<div style=\"display: none;\" id=\"hidden-div-gpt-ad-1484122037505-8\" class=\"[ js-hideable ]\"><\/div>\n<p><script>\n    if (ads_screenwidth < 780 ) {\n        var inline = document.createElement(\"div\");\n        var ad = document.createElement(\"div\");\n\n        ad.id = 'div-gpt-ad-1484122037505-8';\n        ad.style.height=\"250px\";\n        ad.style.width=\"300px;\";\n\n        inline.appendChild(ad);\n        inline.className = \"inline\";\n        var placeholder = document.getElementById(\"hidden-div-gpt-ad-1484122037505-8\");\n        placeholder.parentNode.replaceChild(inline, placeholder);\n        (function () {\n            googletag.cmd.push(function () {\n                googletag.display('div-gpt-ad-1484122037505-8');\n            });\n        })();\n    }\n<\/script>\n                    <\/div>\n<\/p><\/div>\n<\/p><\/div>\n<h2>Caracter\u00edsticas cl\u00ednicas del pseudoxantoma el\u00e1stico<\/h2>\n<p>Las personas con PXE tienen una piel distintiva <span class=\"term\" data-term-id=\"2115\">lesiones<\/span>, cambios en los vasos sangu\u00edneos y anomal\u00edas oculares.  Estos pueden variar en <span class=\"term\" data-term-id=\"69\">distribuci\u00f3n<\/span> y severidad<\/p>\n<h3>Lesiones de la piel<\/h3>\n<p>Las lesiones cut\u00e1neas de PXE son caracter\u00edsticas.  Consisten en peque\u00f1as protuberancias amarillentas en filas o un patr\u00f3n de encaje, que pueden unirse para formar grandes parches.  La piel es suave, laxa y ligeramente arrugada.  Los parches pueden tener un aspecto ligeramente rocoso, lo que se ha descrito como empedrado.<\/p>\n<p>Los sitios comunes afectados en PXE son los lados del cuello, debajo de las clav\u00edculas, las axilas, el abdomen, las ingles, <span class=\"term\" data-term-id=\"889\">perineo<\/span> y muslos.<\/p>\n<p>Estos cambios en la piel pueden <span class=\"term\" data-term-id=\"2343\">desarrollar<\/span> en la infancia, pero generalmente se desarrollan en la edad adulta temprana (antes de los 30 a\u00f1os).  En algunos casos, pueden aparecer por primera vez en la vejez.  Una vez que aparecen, por lo general <span class=\"term\" data-term-id=\"2191\">persistir<\/span> sin cambios indefinidamente.<\/p>\n<p>Pueden ocurrir cambios similares en el paladar blando, dentro de los labios y en el revestimiento del est\u00f3mago. <span class=\"term\" data-term-id=\"2365\">recto<\/span> y <span class=\"term\" data-term-id=\"2391\">vagina<\/span>.<\/p>\n<h3>Coraz\u00f3n y vasos sangu\u00edneos<\/h3>\n<p>El tejido conectivo de <span class=\"term\" data-term-id=\"1962\">arterias<\/span> del coraz\u00f3n y en todo el cuerpo son anormales en el pseudoxantoma el\u00e1stico.  Esto puede provocar dolor en las piernas al caminar (<span class=\"term\" data-term-id=\"1283\">claudicaci\u00f3n<\/span>), accidente cerebrovascular, dolor de coraz\u00f3n (<span class=\"term\" data-term-id=\"1146\">angina de pecho<\/span>) y <span class=\"term\" data-term-id=\"605\">cardiomiopat\u00eda<\/span> (enfermedad del m\u00fasculo card\u00edaco).  El prolapso de la v\u00e1lvula mitral ocurre en alrededor de 5 a 8% de los casos.<\/p>\n<h3>Ojo<\/h3>\n<p><span class=\"term\" data-term-id=\"753\">Vascular<\/span> Las lesiones conocidas como estr\u00edas angioides afectan la retina de la mayor\u00eda de los pacientes con PXE.  Suelen aparecer entre los 20 y los 40 a\u00f1os.  Las rayas angioides pueden provocar <span class=\"term\" data-term-id=\"1759\">progresivo<\/span> falla visual y ocasionalmente ceguera.<\/p>\n<h2>Riesgo obst\u00e9trico asociado con pseudoxantoma el\u00e1stico<\/h2>\n<p>Los pacientes con PXE tienen un mayor riesgo de aborto espont\u00e1neo en los primeros tres meses de embarazo.  Las estr\u00edas se desarrollan durante el embarazo en pr\u00e1cticamente todas las pacientes.<\/p>\n<h2>Seudoxantoma el\u00e1stico adquirido<\/h2>\n<p>Ha habido informes ocasionales de PXE adquirido o de aparici\u00f3n tard\u00eda. <\/p>\n<ul>\n<li>El PXE adquirido se ha asociado con el medicamento penicilamina.<\/li>\n<li>Algunos casos han seguido h\u00edgado <span class=\"term\" data-term-id=\"1793\">trasplante<\/span>.<\/li>\n<li>Tambi\u00e9n se ha informado en pacientes con beta talasemia.<\/li>\n<\/ul>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n                        <!-- \/340621630\/dermnet_mobile_after4sections_300x250' --><\/p>\n<div style=\"display: none;\" id=\"hidden-div-gpt-ad-1484122037505-9\" class=\"[ js-hideable ]\"><\/div>\n<p><script>\n    if (ads_screenwidth < 780 ) {\n        var inline = document.createElement(\"div\");\n        var ad = document.createElement(\"div\");\n\n        ad.id = 'div-gpt-ad-1484122037505-9';\n        ad.style.height=\"250px\";\n        ad.style.width=\"300px;\";\n\n        inline.appendChild(ad);\n        inline.className = \"inline\";\n        var placeholder = document.getElementById(\"hidden-div-gpt-ad-1484122037505-9\");\n        placeholder.parentNode.replaceChild(inline, placeholder);\n        (function () {\n            googletag.cmd.push(function () {\n                googletag.display('div-gpt-ad-1484122037505-9');\n            });\n        })();\n    }\n<\/script>\n                    <\/div>\n<\/p><\/div>\n<\/p><\/div>\n<h2>\u00bfC\u00f3mo se diagnostica el pseudoxantoma el\u00e1stico?<\/h2>\n<p>El diagn\u00f3stico de PXE suele ser sencillo en la piel. <span class=\"term\" data-term-id=\"24\">biopsia<\/span>.  los <span class=\"term\" data-term-id=\"223\">patolog\u00eda<\/span> de PXE es caracter\u00edstico.<\/p>\n<p>Se realizan estudios de im\u00e1genes para determinar su efecto sobre los vasos sangu\u00edneos y el coraz\u00f3n.<\/p>\n<h2>\u00bfCu\u00e1l es el tratamiento para el pseudoxantoma el\u00e1stico?<\/h2>\n<p>El aspecto m\u00e1s importante del tratamiento es asegurar que las complicaciones de <span class=\"term\" data-term-id=\"1068\">vaso sangu\u00edneo<\/span> El especialista apropiado evita o trata r\u00e1pidamente la participaci\u00f3n.  Se recomienda un seguimiento regular con un cirujano vascular especialista y \/ o cardi\u00f3logo.<\/p>\n<ul>\n<li>Actualmente no existe un tratamiento eficaz para las lesiones cut\u00e1neas.<\/li>\n<li>Se ha intentado restringir el calcio en la dieta con alg\u00fan beneficio, pero esto es controvertido.<\/li>\n<li><span class=\"term\" data-term-id=\"1617\">L\u00e1ser<\/span> La fotocoagulaci\u00f3n puede ser \u00fatil para prevenir m\u00e1s hemorragias en la parte posterior del ojo.<\/li>\n<li>Dado que existe el riesgo de transmitir esta afecci\u00f3n a la siguiente generaci\u00f3n, el asesoramiento gen\u00e9tico puede resultar \u00fatil.<\/li>\n<\/ul>\n<\/section>\n","protected":false},"excerpt":{"rendered":"<p>\u00bfQu\u00e9 es el pseudoxantoma el\u00e1stico? Pseudoxantoma el\u00e1stico (PXE) es el nombre que se le da a un grupo de tejido conectivo trastornos que afectan el tejido el\u00e1stico de la piel, vasos sanguineosy los ojos. PXE tambi\u00e9n se conoce como Gronblad-Strandberg s\u00edndrome. Pseudoxantoma el\u00e1stico Pseudoxantoma el\u00e1stico de la fosa antecubital \u00bfCu\u00e1l es la causa del pseudoxantoma [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":10848,"parent":0,"template":"","glossary-cat":[],"class_list":["post-10844","glossary","type-glossary","status-publish","has-post-thumbnail","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v26.2 (Yoast SEO v27.1.1) - https:\/\/yoast.com\/product\/yoast-seo-premium-wordpress\/ -->\n<title>Pseudoxantoma el\u00e1stico - Dermatly.com - El sitio de tu piel<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/dermatly.com\/it\/pseudoxantoma-elastico\/\" \/>\n<meta property=\"og:locale\" content=\"it_IT\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Pseudoxantoma el\u00e1stico\" \/>\n<meta property=\"og:description\" content=\"\u00bfQu\u00e9 es el pseudoxantoma el\u00e1stico? Pseudoxantoma el\u00e1stico (PXE) es el nombre que se le da a un grupo de tejido conectivo trastornos que afectan el tejido el\u00e1stico de la piel, vasos sanguineosy los ojos. PXE tambi\u00e9n se conoce como Gronblad-Strandberg s\u00edndrome. Pseudoxantoma el\u00e1stico Pseudoxantoma el\u00e1stico de la fosa antecubital \u00bfCu\u00e1l es la causa del pseudoxantoma [&hellip;]\" \/>\n<meta property=\"og:url\" content=\"https:\/\/dermatly.com\/it\/pseudoxantoma-elastico\/\" \/>\n<meta property=\"og:site_name\" content=\"Dermatly.com - El sitio de tu piel\" \/>\n<meta property=\"article:modified_time\" content=\"2026-03-10T09:55:48+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/pxe__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-4124014.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"294\" \/>\n\t<meta property=\"og:image:height\" content=\"222\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Tempo di lettura stimato\" \/>\n\t<meta name=\"twitter:data1\" content=\"4 minuti\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/dermatly.com\/pseudoxantoma-elastico\/\",\"url\":\"https:\/\/dermatly.com\/pseudoxantoma-elastico\/\",\"name\":\"Pseudoxantoma el\u00e1stico - Dermatly.com - El sitio de tu piel\",\"isPartOf\":{\"@id\":\"https:\/\/dermatly.com\/#website\"},\"primaryImageOfPage\":{\"@id\":\"https:\/\/dermatly.com\/pseudoxantoma-elastico\/#primaryimage\"},\"image\":{\"@id\":\"https:\/\/dermatly.com\/pseudoxantoma-elastico\/#primaryimage\"},\"thumbnailUrl\":\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/pxe__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-4124014.jpg\",\"datePublished\":\"2020-11-18T03:41:29+00:00\",\"dateModified\":\"2026-03-10T09:55:48+00:00\",\"breadcrumb\":{\"@id\":\"https:\/\/dermatly.com\/pseudoxantoma-elastico\/#breadcrumb\"},\"inLanguage\":\"it-IT\",\"potentialAction\":[{\"@type\":\"ReadAction\",\"target\":[\"https:\/\/dermatly.com\/pseudoxantoma-elastico\/\"]}]},{\"@type\":\"ImageObject\",\"inLanguage\":\"it-IT\",\"@id\":\"https:\/\/dermatly.com\/pseudoxantoma-elastico\/#primaryimage\",\"url\":\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/pxe__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-4124014.jpg\",\"contentUrl\":\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/pxe__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-4124014.jpg\",\"width\":294,\"height\":222,\"caption\":\"pxe__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-4124014-1599199-jpg-9137503\"},{\"@type\":\"BreadcrumbList\",\"@id\":\"https:\/\/dermatly.com\/pseudoxantoma-elastico\/#breadcrumb\",\"itemListElement\":[{\"@type\":\"ListItem\",\"position\":1,\"name\":\"Home\",\"item\":\"https:\/\/dermatly.com\/\"},{\"@type\":\"ListItem\",\"position\":2,\"name\":\"Glossary\",\"item\":\"https:\/\/dermatly.com\/glossary\/\"},{\"@type\":\"ListItem\",\"position\":3,\"name\":\"Pseudoxantoma el\u00e1stico\"}]},{\"@type\":\"WebSite\",\"@id\":\"https:\/\/dermatly.com\/#website\",\"url\":\"https:\/\/dermatly.com\/\",\"name\":\"Dermatly.com - El sitio de tu piel\",\"description\":\"Dermatolog\u00eda y lesiones de la piel\",\"publisher\":{\"@id\":\"https:\/\/dermatly.com\/#organization\"},\"potentialAction\":[{\"@type\":\"SearchAction\",\"target\":{\"@type\":\"EntryPoint\",\"urlTemplate\":\"https:\/\/dermatly.com\/?s={search_term_string}\"},\"query-input\":{\"@type\":\"PropertyValueSpecification\",\"valueRequired\":true,\"valueName\":\"search_term_string\"}}],\"inLanguage\":\"it-IT\"},{\"@type\":\"Organization\",\"@id\":\"https:\/\/dermatly.com\/#organization\",\"name\":\"dermatly.com\",\"url\":\"https:\/\/dermatly.com\/\",\"logo\":{\"@type\":\"ImageObject\",\"inLanguage\":\"it-IT\",\"@id\":\"https:\/\/dermatly.com\/#\/schema\/logo\/image\/\",\"url\":\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/dermatly-logo.png\",\"contentUrl\":\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/dermatly-logo.png\",\"width\":448,\"height\":110,\"caption\":\"dermatly.com\"},\"image\":{\"@id\":\"https:\/\/dermatly.com\/#\/schema\/logo\/image\/\"}}]}<\/script>\n<!-- \/ Yoast SEO Premium plugin. -->","yoast_head_json":{"title":"Pseudoxantoma elastico - Dermatly.com - Il sito della tua pelle","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/dermatly.com\/it\/pseudoxantoma-elastico\/","og_locale":"it_IT","og_type":"article","og_title":"Pseudoxantoma el\u00e1stico","og_description":"\u00bfQu\u00e9 es el pseudoxantoma el\u00e1stico? 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