{"id":11593,"date":"2020-11-19T00:26:26","date_gmt":"2020-11-19T00:26:26","guid":{"rendered":"https:\/\/dermatly.com\/penfigo-iga\/"},"modified":"2026-03-10T16:45:48","modified_gmt":"2026-03-10T16:45:48","slug":"pemphigo-iga","status":"publish","type":"glossary","link":"https:\/\/dermatly.com\/de\/penfigo-iga\/","title":{"rendered":"IgA-Pemphigus"},"content":{"rendered":"<section class=\"textBlock\">\n<h1>Definici\u00f3n y Subtipos del P\u00e9nfigo IgA<\/h1>\n<p>El P\u00e9nfigo IgA, tambi\u00e9n conocido como <span class=\"term\" data-term-id=\"427\">inmunoglobulina<\/span> Un p\u00e9nfigo, es un trastorno cut\u00e1neo <span class=\"term\" data-term-id=\"691\">autoinmune<\/span> caracterizado por la formaci\u00f3n de ampollas. Esta afecci\u00f3n se denomina tambi\u00e9n <span class=\"term\" data-term-id=\"2104\">intercelular<\/span> IgA <span class=\"term\" data-term-id=\"60\">dermatosis<\/span>, entre otras designaciones.<\/p>\n<p>Hist\u00f3ricamente, el p\u00e9nfigo IgA se ha dividido en dos subtipos principales:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"1056\">Subcorneal<\/span> <span class=\"term\" data-term-id=\"792\">pustuloso<\/span> tipo de dermatosis (SPD).<\/li>\n<li><span class=\"term\" data-term-id=\"1038\">Intraepid\u00e9rmico<\/span> <span class=\"term\" data-term-id=\"443\">neutrof\u00edlico<\/span> (IEN) tipo.<\/li>\n<\/ul>\n<p>No obstante, una clasificaci\u00f3n m\u00e1s reciente ampl\u00eda esta divisi\u00f3n a cinco subtipos de p\u00e9nfigo IgA, que incluyen:<\/p>\n<ul>\n<li>P\u00e9nfigo de IgA tipo SPD.<\/li>\n<li>P\u00e9nfigo IEN tipo IgA.<\/li>\n<li>IgA-p\u00e9nfigo vegetativo.<\/li>\n<li>P\u00e9nfigo IgA vulgar.<\/li>\n<li>P\u00e9nfigo IgA no clasificado.<\/li>\n<\/ul>\n<h2>\u00bfQui\u00e9n Desarrolla el P\u00e9nfigo de IgA?<\/h2>\n<p>El p\u00e9nfigo IgA es una enfermedad extremadamente rara. Su incidencia es mayor en personas de mediana edad y ancianos, aunque puede manifestarse en cualquier grupo etario; se ha documentado un caso en un lactante de un mes de edad.<\/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>Manifestaciones Cl\u00ednicas del P\u00e9nfigo IgA<\/h2>\n<p>Las caracter\u00edsticas cl\u00ednicas t\u00edpicas del p\u00e9nfigo IgA incluyen la aparici\u00f3n de ampollas, <span class=\"term\" data-term-id=\"1016\">p\u00fastulas<\/span>, <span class=\"term\" data-term-id=\"90\">eritema<\/span>, <span class=\"term\" data-term-id=\"536\">erosiones<\/span> y <span class=\"term\" data-term-id=\"2115\">lesiones<\/span> vegetantes.<\/p>\n<ul>\n<li>Los signos iniciales se manifiestan como <span class=\"term\" data-term-id=\"1627\">fl\u00e1cidas<\/span> <span class=\"term\" data-term-id=\"1028\">ves\u00edculas<\/span> y p\u00fastulas.<\/li>\n<li>Estas ves\u00edculas y p\u00fastulas secundariamente se rompen, dando lugar a erosiones y <span class=\"term\" data-term-id=\"2013\">costrosas<\/span> <span class=\"term\" data-term-id=\"368\">placas<\/span>.<\/li>\n<li>Es com\u00fan que la <span class=\"term\" data-term-id=\"704\">erupci\u00f3n<\/span> adopte un patr\u00f3n <span class=\"term\" data-term-id=\"9\">anular<\/span> o semicircular.<\/li>\n<\/ul>\n<p>El p\u00e9nfigo IgA tiende a afectar predominantemente el tronco, las extremidades superiores e inferiores, las <span class=\"term\" data-term-id=\"629\">axilas<\/span> y la ingle. La afectaci\u00f3n de la superficie <span class=\"term\" data-term-id=\"1018\">mucosa<\/span> es un hallazgo infrecuente en esta patolog\u00eda.<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h3 class=\"colour--primary\">Visualizaci\u00f3n del P\u00e9nfigo IgA<\/h3>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n                <img decoding=\"async\" src=\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/iga-pv__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsInkiLDEwN10.jpg\" alt=\"Imagen representativa de P\u00e9nfigo IgA mostrando lesiones cut\u00e1neas.\">\n            <\/div>\n<p class=\"p-small\">P\u00e9nfigo IgA<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<h2>Causas Subyacentes del P\u00e9nfigo IgA<\/h2>\n<p>La etiolog\u00eda precisa del p\u00e9nfigo IgA permanece sin esclarecer completamente.<\/p>\n<ul>\n<li>Los <span class=\"term\" data-term-id=\"352\">autoanticuerpos<\/span> de tipo IgA se dirigen contra las <span class=\"term\" data-term-id=\"1031\">desmogle\u00ednas<\/span> o desmocolinas, las prote\u00ednas esenciales responsables de la <span class=\"term\" data-term-id=\"822\">adhesi\u00f3n<\/span> celular.<\/li>\n<li>Espec\u00edficamente en el p\u00e9nfigo IgA tipo SPD, el <span class=\"term\" data-term-id=\"701\">autoant\u00edgeno<\/span> identificado es la desmocolina 1, una de las cadherinas desmos\u00f3micas, que son <span class=\"term\" data-term-id=\"1639\">glicoprote\u00ednas<\/span> cruciales para mantener la integridad estructural celular.<\/li>\n<li>Se ha determinado que, en una proporci\u00f3n de los casos de p\u00e9nfigo IgA tipo IEN, los <span class=\"term\" data-term-id=\"679\">autoant\u00edgenos<\/span> involucrados son la <span class=\"term\" data-term-id=\"1030\">desmogle\u00edna<\/span> 1, la desmogle\u00edna 3 y otra mol\u00e9cula transmembrana.<\/li>\n<\/ul>\n<p>Comprender estos mecanismos autoinmunitarios es fundamental para el diagn\u00f3stico y desarrollo de tratamientos efectivos contra esta rara enfermedad de la piel.<\/p>\n<\/section>\n<ul>\n<li>No se especifica la <span class=\"term\" data-term-id=\"2224\">prote\u00edna<\/span>.<\/li>\n<\/ul>\n<h2>Diagn\u00f3stico Diferencial del P\u00e9nfigo IgA<\/h2>\n<p>Otras afecciones cut\u00e1neas que deben considerarse en el diagn\u00f3stico diferencial del p\u00e9nfigo IgA incluyen:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"937\">Infecci\u00f3n<\/span> <span class=\"term\" data-term-id=\"145\">bacteriana<\/span> de la piel<\/li>\n<li><span class=\"term\" data-term-id=\"53\">Dermatitis<\/span> herpetiforme<\/li>\n<li>P\u00e9nfigo foli\u00e1ceo<\/li>\n<li>Foliculitis pustulosa <span class=\"term\" data-term-id=\"449\">eosinof\u00edlica<\/span><\/li>\n<li>Dermatosis pustulosa subcorneal.<\/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>Procedimiento Diagn\u00f3stico del P\u00e9nfigo IgA<\/h2>\n<p>Una enfermedad ampollosa <span class=\"term\" data-term-id=\"1251\">autoinflamatoria<\/span> o <span class=\"term\" data-term-id=\"1251\">autoinmune<\/span> debe sospecharse cl\u00ednicamente por sus caracter\u00edsticas, especialmente cuando la erupci\u00f3n no responde al tratamiento con antibi\u00f3ticos.<\/p>\n<p>Establecer el diagn\u00f3stico de p\u00e9nfigo IgA requiere la realizaci\u00f3n de una <span class=\"term\" data-term-id=\"24\">biopsia<\/span> de piel para el an\u00e1lisis <span class=\"term\" data-term-id=\"128\">histol\u00f3gico<\/span> y la aplicaci\u00f3n de pruebas de inmunofluorescencia directa.<\/p>\n<ul>\n<li>Los hallazgos en la biopsia de piel de p\u00e9nfigo IgA suelen mostrar una ampolla intraepid\u00e9rmica. Se observa un <span class=\"term\" data-term-id=\"52\">infiltrado<\/span> <span class=\"term\" data-term-id=\"490\">inflamatorio<\/span> <span class=\"term\" data-term-id=\"541\">d\u00e9rmico<\/span>, acompa\u00f1ado de <span class=\"term\" data-term-id=\"543\">infiltraci\u00f3n<\/span> intraepid\u00e9rmica de <span class=\"term\" data-term-id=\"441\">neutr\u00f3filos<\/span>.<\/li>\n<li>La <span class=\"term\" data-term-id=\"142\">inmunofluorescencia<\/span> directa realizada sobre una biopsia de piel de apariencia sana, cercana al \u00e1rea afectada, revela el **dep\u00f3sito (o <span class=\"term\" data-term-id=\"370\">declaraci\u00f3n<\/span>)** de inmunoglobulina A (IgA) en las membranas celulares de los <span class=\"term\" data-term-id=\"917\">queratinocitos<\/span> <span class=\"term\" data-term-id=\"846\">epid\u00e9rmicos<\/span>.<\/li>\n<\/ul>\n<p>Adem\u00e1s, la prueba de inmunofluorescencia indirecta realizada sobre el <span class=\"term\" data-term-id=\"1430\">suero<\/span> puede evidenciar la presencia de <span class=\"term\" data-term-id=\"426\">anticuerpos<\/span> anti-queratinocitos de superficie celular de tipo IgA. Tambi\u00e9n se han documentado casos con anticuerpos anti-queratinocitos de superficie celular de tipo inmunoglobulina G (IgG).<\/p>\n<h2>Opciones de Tratamiento para el P\u00e9nfigo IgA<\/h2>\n<p>Los pilares terap\u00e9uticos fundamentales para manejar el p\u00e9nfigo IgA son los corticosteroides <span class=\"term\" data-term-id=\"380\">sist\u00e9micos<\/span> y la dapsona.<\/p>\n<p>Puesto que se han reportado relativamente pocos casos de p\u00e9nfigo IgA, el r\u00e9gimen de tratamiento \u00f3ptimo sigue siendo objeto de estudio. Sin embargo, existen otras opciones farmacol\u00f3gicas que merecen ser consideradas:<\/p>\n<ul>\n<li>Colchicina<\/li>\n<li>Micofenolato de mofetilo<\/li>\n<li>Isotretino\u00edna<\/li>\n<li>Acitretina<\/li>\n<li>Adalimumab.<\/li>\n<\/ul>\n<\/section>\n","protected":false},"excerpt":{"rendered":"<p>Definici\u00f3n y Subtipos del P\u00e9nfigo IgA El P\u00e9nfigo IgA, tambi\u00e9n conocido como inmunoglobulina Un p\u00e9nfigo, es un trastorno cut\u00e1neo autoinmune caracterizado por la formaci\u00f3n de ampollas. Esta afecci\u00f3n se denomina tambi\u00e9n intercelular IgA dermatosis, entre otras designaciones. Hist\u00f3ricamente, el p\u00e9nfigo IgA se ha dividido en dos subtipos principales: Subcorneal pustuloso tipo de dermatosis (SPD). Intraepid\u00e9rmico [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":11595,"parent":0,"template":"","glossary-cat":[],"class_list":["post-11593","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>P\u00e9nfigo IgA - 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\/de\/pemphigo-iga\/\" \/>\n<meta property=\"og:locale\" content=\"de_DE\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"P\u00e9nfigo IgA\" \/>\n<meta property=\"og:description\" content=\"Definici\u00f3n y Subtipos del P\u00e9nfigo IgA El P\u00e9nfigo IgA, tambi\u00e9n conocido como inmunoglobulina Un p\u00e9nfigo, es un trastorno cut\u00e1neo autoinmune caracterizado por la formaci\u00f3n de ampollas. Esta afecci\u00f3n se denomina tambi\u00e9n intercelular IgA dermatosis, entre otras designaciones. Hist\u00f3ricamente, el p\u00e9nfigo IgA se ha dividido en dos subtipos principales: Subcorneal pustuloso tipo de dermatosis (SPD). 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Esta afecci\u00f3n se denomina tambi\u00e9n intercelular IgA dermatosis, entre otras designaciones. Hist\u00f3ricamente, el p\u00e9nfigo IgA se ha dividido en dos subtipos principales: Subcorneal pustuloso tipo de dermatosis (SPD). 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