{"id":9429,"date":"2020-04-28T05:34:44","date_gmt":"2020-04-28T08:34:44","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/wiki\/patologia-de-la-enfermedad-de-la-piel-relacionada-con-igg4\/"},"modified":"2020-04-30T21:34:28","modified_gmt":"2020-05-01T00:34:28","slug":"pathology-of-skin-disease-related-with-igg4-2","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/en\/wiki\/patologia-de-la-enfermedad-de-la-piel-relacionada-con-igg4\/","title":{"rendered":"IgG4-related skin disease pathology"},"content":{"rendered":"<p><\/p><div id=\"ez-toc-container\" class=\"ez-toc-v2_0_88 counter-hierarchy ez-toc-counter ez-toc-grey ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Contents<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Toggle Table of Content\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewbox=\"0 0 24 24\" version=\"1.2\" baseprofile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 eztoc-toggle-hide-by-default' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/patologia-de-la-enfermedad-de-la-piel-relacionada-con-igg4\/#Introduccion\" >Introduction<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/patologia-de-la-enfermedad-de-la-piel-relacionada-con-igg4\/#Histologia-de-la-enfermedad-cutanea-relacionada-con-IgG4\" >Histology of IgG4-related skin disease<\/a><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/patologia-de-la-enfermedad-de-la-piel-relacionada-con-igg4\/#Patologia-de-la-enfermedad-de-la-piel-relacionada-con-IgG4\" >IgG4-related skin disease pathology<\/a><\/li><\/ul><\/li><\/ul><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/patologia-de-la-enfermedad-de-la-piel-relacionada-con-igg4\/#Tinciones-especiales-de-la-enfermedad-de-la-piel-relacionada-con-IgG4\" >Special stains for IgG4-related skin disease<\/a><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/patologia-de-la-enfermedad-de-la-piel-relacionada-con-igg4\/#Enfermedad-cutanea-relacionada-con-IgG4-manchas-especiales\" >IgG4-related skin disease: special spots<\/a><\/li><\/ul><\/li><\/ul><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/patologia-de-la-enfermedad-de-la-piel-relacionada-con-igg4\/#Clasificacion-de-la-enfermedad-cutanea-relacionada-con-IgG4\" >Classification of IgG4-related skin disease<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/patologia-de-la-enfermedad-de-la-piel-relacionada-con-igg4\/#Diagnostico-diferencial-de-la-enfermedad-cutanea-relacionada-con-IgG4\" >Differential diagnosis of IgG4-related skin disease<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"Introduccion\"><\/span>Introduction<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>IgG4-related disease is a recently described entity. It is characterized by:<\/p>\n<ul>\n<li>High levels of circulating IgG4 (&gt; 135 mg \/ dL) in 60 to 70% of patients<\/li>\n<li>Tissue <span class=\"term\" data-term-id=\"543\">infiltration<\/span> IgG4 + <span class=\"term\" data-term-id=\"1513\">plasma<\/span> cells<\/li>\n<li>Fibroinflammatory change in various organs such as the pancreas, saliva, <span class=\"term\" data-term-id=\"1434\">lacrimal<\/span>etc.<\/li>\n<li>Surprising <span class=\"term\" data-term-id=\"474\">histological<\/span> similarities between organs.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"Histologia-de-la-enfermedad-cutanea-relacionada-con-IgG4\"><\/span>\n<span class=\"term\" data-term-id=\"128\">Histology<\/span> of IgG4-related skin disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<ul>\n<li>Dense lymphoplasmacytic <span class=\"term\" data-term-id=\"541\">infiltrate<\/span> (figures 1,2)<\/li>\n<li>Storiform <span class=\"term\" data-term-id=\"102\">fibrosis<\/span> (fibrosis in a cartwheel pattern, figure 3)<\/li>\n<li>Moderate to moderate weave <span class=\"term\" data-term-id=\"83\">eosinophilia<\/span> (Figure 4)<\/li>\n<\/ul>\n<p>In general, the minimum diagnostic for most tissues is 30 to 50 IgG4-positive cells per high-power field. However, in some organs or tissues, eg kidney, only 10 IgG4 positive plasma cells per high power field may be sufficient.<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Patologia-de-la-enfermedad-de-la-piel-relacionada-con-IgG4\"><\/span>IgG4-related skin disease pathology<span class=\"ez-toc-section-end\"><\/span><\/h5>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='igg4-related-1__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-3605057-3789547' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/igg4-related-1__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-3605057.jpg'>\n<\/div>\n<p class=\"p-small\">Figure 1<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='igg4-related-2__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-9909157-5878833' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/igg4-related-2__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-9909157.jpg'>\n<\/div>\n<p class=\"p-small\">Figure 2<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='igg4-related-3__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-7822586-6690863' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/igg4-related-3__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-7822586.jpg'>\n<\/div>\n<p class=\"p-small\">figure 3<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='igg4-related-4__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-3393964-1543780' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/igg4-related-4__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-3393964.jpg'>\n<\/div>\n<p class=\"p-small\">Figure 4<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"Tinciones-especiales-de-la-enfermedad-de-la-piel-relacionada-con-IgG4\"><\/span>Special stains for IgG4-related skin disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<ul>\n<li>The CD3 stain illustrates the dense population of <span class=\"term\" data-term-id=\"419\">T <span class=\"term\" data-term-id=\"421\">lymphocytes<\/span><\/span>  (Figure 5)<\/li>\n<li>CD20 staining illustrates the population of plasma cells \/ <span class=\"term\" data-term-id=\"430\">B cells<\/span> (figure 6)<\/li>\n<li>The anti IgG4 stain that looks brown shows an impressive&gt; 250 IgG4 + B cells in the high power field (Figure 7)<\/li>\n<\/ul>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Enfermedad-cutanea-relacionada-con-IgG4-manchas-especiales\"><\/span>IgG4-related skin disease: special spots<span class=\"ez-toc-section-end\"><\/span><\/h5>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='igg4-related-5__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-1945183-1810757' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/igg4-related-5__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-1945183.jpg'>\n<\/div>\n<p class=\"p-small\">Figure 5<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='igg4-related-6__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-4793320-9122117' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/igg4-related-6__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-4793320.jpg'>\n<\/div>\n<p class=\"p-small\">Figure 6<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='igg4-related-7__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-5662114-5369549' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/igg4-related-7__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-5662114.jpg'>\n<\/div>\n<p class=\"p-small\">Figure 7<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<h2><span class=\"ez-toc-section\" id=\"Clasificacion-de-la-enfermedad-cutanea-relacionada-con-IgG4\"><\/span>Classification of IgG4-related skin disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Yokura et al (2014) proposed a classification of IgG4-related skin disease that divides it into <span class=\"term\" data-term-id=\"949\">primary<\/span>, mass-forming lesions due to direct infiltration of plasma cells, and secondary lesions due to IgG4 mediation <span class=\"term\" data-term-id=\"148\">inflammation<\/span> through secondary mechanisms.<\/p>\n<p>Primary injuries include:<\/p>\n<ul>\n<li>\n<span class=\"term\" data-term-id=\"47\">Cutaneous<\/span> plasmacytosis<\/li>\n<li>Pseudolymphoma and angiolymphoid <span class=\"term\" data-term-id=\"136\">hyperplasia<\/span> with eosinophilia<\/li>\n<li>Mikulicz disease (inflammation of the lacrimal and salivary glands).<\/li>\n<\/ul>\n<p>Secondary injuries include:<\/p>\n<ul>\n<li>Lesions similar to psoriasis<\/li>\n<li>Unspecified <span class=\"term\" data-term-id=\"938\">maculopapular<\/span> or <span class=\"term\" data-term-id=\"967\">erythematous<\/span> injuries<\/li>\n<li>Hypergammaglobulinemia, <span class=\"term\" data-term-id=\"250\">purple<\/span> and <span class=\"term\" data-term-id=\"293\">urticarial<\/span> <span class=\"term\" data-term-id=\"1091\">vasculitis<\/span>.<\/li>\n<li>\n<span class=\"term\" data-term-id=\"765\">Ischemic<\/span> <span class=\"term\" data-term-id=\"1267\">digit<\/span>\n<\/li>\n<\/ul>\n<p>The proposed diagnostic criteria on histology for skin disease are as follows:<\/p>\n<ul>\n<li>IgG4-related primary skin disease<\/li>\n<li>Marked <span class=\"term\" data-term-id=\"420\">lymphocyte<\/span> and plasmacyte infiltration, IgG4 + \/ IgG +&gt; 40%<\/li>\n<li>No. of IgG4 + cells per high power field&gt; 10.<\/li>\n<\/ul>\n<p>IgG4-related secondary skin disease<\/p>\n<ul>\n<li>Infiltration of plasma cells with IgG4 + \/ IgG +&gt; 40% and \/ or <span class=\"term\" data-term-id=\"228\">perivascular<\/span> IgG4 <span class=\"term\" data-term-id=\"370\">statement<\/span>\n<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"Diagnostico-diferencial-de-la-enfermedad-cutanea-relacionada-con-IgG4\"><\/span>\n<span class=\"term\" data-term-id=\"813\">Differential diagnosis<\/span> of IgG4-related skin disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Differential diagnosis of <span class=\"term\" data-term-id=\"427\">immunoglobulin<\/span> G4 (IgG4-RD) -related disease is extensive and depends on the specific site of involvement and clinical presentation.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>Introducci\u00f3n La enfermedad relacionada con IgG4 es una entidad recientemente descrita. Se caracteriza por: Altos niveles de IgG4 circulante (&gt; 135 mg \/ dL) en 60 a 70% de los&#8230;<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[204],"tags":[205],"class_list":["post-9429","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones","tag-patologia"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/9429","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/comments?post=9429"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/9429\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=9429"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=9429"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=9429"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}