{"id":8019,"date":"2020-04-26T20:04:15","date_gmt":"2020-04-26T23:04:15","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/displasia-ectodermica\/"},"modified":"2020-04-27T14:57:55","modified_gmt":"2020-04-27T17:57:55","slug":"ectodermal-dysplasia","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/en\/wiki\/displasia-ectodermica\/","title":{"rendered":"Ectodermal dysplasia"},"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\/displasia-ectodermica\/#Que-es-ectodermico-displasia\" >What is ectodermal dysplasia?<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/displasia-ectodermica\/#Clasificacion-de-la-displasia-ectodermica\" >Classification of ectodermal dysplasia<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/displasia-ectodermica\/#%C2%BFCual-es-la-causa-de-la-displasia-ectodermica\" >What is the cause of ectodermal dysplasia?<\/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-4\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/displasia-ectodermica\/#Genetica-de-la-displasia-ectodermica\" >Genetics of Ectodermal Dysplasia *<\/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-5\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/displasia-ectodermica\/#%C2%BFCuales-son-los-signos-y-sintomas-de-la-displasia-ectodermica\" >What are the signs and symptoms of ectodermal dysplasia?<\/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-6\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/displasia-ectodermica\/#Displasia-ectodermica-anhidrotica\" >Anhydrotic ectodermal dysplasia<\/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-7\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/displasia-ectodermica\/#%C2%BFCual-es-el-tratamiento-para-la-displasia-ectodermica\" >What is the treatment for ectodermal dysplasia?<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"Que-es-ectodermico-displasia\"><\/span>What is it <span class=\"term\" data-term-id=\"375\">ectodermal<\/span> <span class=\"term\" data-term-id=\"73\"><span class=\"term\" data-term-id=\"1828\">dysplasia<\/span><\/span>?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Ectodermal dysplasia is a large group of inherited disorders characterized by a <span class=\"term\" data-term-id=\"949\">primary<\/span> defect in <span class=\"term\" data-term-id=\"122\">hair<\/span>teeth <span class=\"term\" data-term-id=\"193\">nail<\/span> or function of the sweat glands, in addition to another abnormality in any tissue of ectodermal origin. These are ears, eyes, lips, <span class=\"term\" data-term-id=\"188\">mucous<\/span> membranes of the mouth or nose, and the central nervous system.<\/p>\n<p>The ectoderm is the outermost layer of cells in embryonic development and contributes to the formation of many parts of the body, including all of those described above. Ectodermal dysplasia occurs when the ectoderm of certain areas does not develop normally. All ectodermal dysplasias are present from birth and are not<span class=\"term\" data-term-id=\"1759\">progressive<\/span>.<\/p>\n<h3><span class=\"ez-toc-section\" id=\"Clasificacion-de-la-displasia-ectodermica\"><\/span>Classification of ectodermal dysplasia<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<p>Currently, there are about 150 different types of ectodermal dysplasias. In an attempt to classify them, different subgroups are created according to the presence or absence of the four defects of primary ectodermal dysplasia (ED):<\/p>\n<ul>\n<li>ED1: Trichodysplasia (capillary dysplasia)<\/li>\n<li>ED2: dental dysplasia<\/li>\n<li>ED3: Onychodysplasia (nail dysplasia)<\/li>\n<li>ED4: Dyshidrosis (dysplasia of sweat glands)<\/li>\n<\/ul>\n<p>Based on the above, the 150 different types of ectodermal dysplasias are classified into one of the following subgroups formed by the primary ED defects:<\/p>\n<ul>\n<li>Subgroup 1-2-3-4<\/li>\n<li>Subgroup 1-2-3<\/li>\n<li>Subgroup 1-2-4<\/li>\n<li>Subgroup 1-2<\/li>\n<li>Subgroup 1-3<\/li>\n<li>Subgroup 1-4<\/li>\n<li>Subgroup 2-3-4<\/li>\n<li>Subgroup 2-3<\/li>\n<li>Subgroup 2-4<\/li>\n<li>Subgroup 3<\/li>\n<li>Subgroup 4<\/li>\n<\/ul>\n<p>The most common ectodermal dysplasias are hypohydrotic (anhydrotic) ED belonging to subgroup 1-2-3-4 and hydrotic ED belonging to subgroup 1-2-3.<\/p>\n<p>The three most recognized ectodermal dysplasia syndromes fall in subgroup 1-2-3-4, as they show characteristics of the four primary ED defects. They are:<\/p>\n<ul>\n<li>Electrodactyly-ED-<span class=\"term\" data-term-id=\"1834\">cleft<\/span> <span class=\"term\" data-term-id=\"350\">syndrome<\/span>\n<\/li>\n<li>Rapp-Hodgkin Hypohydrotic ED<\/li>\n<li>Ankyloblepharon, ectodermal defects, <span class=\"term\" data-term-id=\"1835\">cleft<\/span> lip \/ palate (AEC) or Hay-Wells syndrome<\/li>\n<\/ul>\n<p><span class=\"term\" data-term-id=\"1592\">Prenatal<\/span> Tests are available for ectodermal dysplasia syndromes at some centers.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-la-causa-de-la-displasia-ectodermica\"><\/span>What is the cause of ectodermal dysplasia?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The different types of ectodermal dysplasia are caused by <span class=\"term\" data-term-id=\"870\">mutation<\/span> or removal of certain <span class=\"term\" data-term-id=\"775\">genes<\/span> located in different <span class=\"term\" data-term-id=\"1272\">chromosomes<\/span>. Because ectodermal dysplasias are caused by a <span class=\"term\" data-term-id=\"558\">genetic<\/span> default, they can be inherited or transmitted by the family line. In some cases, they can occur in people with no family history of the condition, in which case a <span class=\"term\" data-term-id=\"746\">de novo<\/span> The mutation has occurred.<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Genetica-de-la-displasia-ectodermica\"><\/span>Genetics of Ectodermal Dysplasia *<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='ectodermal-dysplasia__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildbd-3433220-2916614' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/Ectodermal-dysplasia__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDBd-3433220.png'>\n<\/div>\n<p class=\"p-small\">Ectodermal dysplasia<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<p style=\"font-size: 10px;\">* Image courtesy of Genetics 4 Medics<\/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<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCuales-son-los-signos-y-sintomas-de-la-displasia-ectodermica\"><\/span>What are the signs and symptoms of ectodermal dysplasia?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The signs and symptoms of ectodermal dysplasia differ markedly between different types and depend on the structures affected. Signs and symptoms are generally not apparent in newborns and cannot be detected until infancy or childhood.<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Displasia-ectodermica-anhidrotica\"><\/span>Anhydrotic ectodermal dysplasia<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='anhidrectdysp__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisinkildm2xq-4025231-6230609' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/anhidrectdysp__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsInkiLDM2XQ-4025231.jpg'>\n<\/div>\n<p class=\"p-small\">Anhydrotic ectodermal dysplasia<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<p>Ectodermal dysplasia generally affects the four organs involved primarily in the following ways:<\/p>\n<table class=\"borders\">\n<tbody>\n<tr>\n<th>Organ affected<\/th>\n<th>Characteristics<\/th>\n<\/tr>\n<tr>\n<td>Hair<\/td>\n<td>\n<ul>\n<li>The scalp and body hair can be thin, scattered, and light in color.<\/li>\n<li>Hair can be thick, excessively brittle, curly, or even twisted<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n<tr>\n<td>Nail<\/td>\n<td>\n<ul>\n<li>Fingernails and toenails may be thick, abnormally shaped, discolored, striated, slow growing, or brittle<\/li>\n<li>Sometimes the nails can be absent<\/li>\n<li>Cuticles may be prone to <span class=\"term\" data-term-id=\"145\">infection<\/span>\n<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n<tr>\n<td>Teeth<\/td>\n<td>\n<ul>\n<li>Abnormal development of teeth resulting in missing teeth or growth of pin-shaped or pointed teeth<\/li>\n<li>Tooth enamel is also defective<\/li>\n<li>Dental treatment is necessary and children up to two years old may need prosthetics<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n<tr>\n<td>Sweat glands<\/td>\n<td>\n<ul>\n<li>\n<span class=\"term\" data-term-id=\"844\">Eccrine<\/span> sweat glands may be absent or too few for sweat glands to function abnormally or not to work<\/li>\n<li>Without normal sweat production, the body cannot regulate temperature properly<\/li>\n<li>Children can experience <span class=\"term\" data-term-id=\"805\">recurrent<\/span> tall <span class=\"term\" data-term-id=\"1176\">fever<\/span> that can lead to seizures and <span class=\"term\" data-term-id=\"402\">neurological <\/span>problems<\/li>\n<li>Overheating is a common problem, particularly in warmer climates.<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>Other signs and symptoms include:<\/p>\n<ul>\n<li>Slightly <span class=\"term\" data-term-id=\"1520\">pigmented<\/span> skin, in some cases red or brown <span class=\"term\" data-term-id=\"231\">pigment<\/span> may be present. The skin may be thick on the palms and soles and is prone to cracking, bleeding, and infection.<\/li>\n<li>The skin may be dry and prone to rashes and infections.<\/li>\n<li>Dry eyes occur due to lack of tears. <span class=\"term\" data-term-id=\"927\">waterfalls<\/span> and visual defects can also occur.<\/li>\n<li>Abnormal ear development can cause hearing problems.<\/li>\n<li>Cleft palate \/ lip.<\/li>\n<li>Lack of fingers and toes (<span class=\"term\" data-term-id=\"1268\">digits<\/span>)<\/li>\n<li>Respiratory infections due to a lack of normal protective secretions from the mouth and nose.<\/li>\n<li>Smelly Nasal <span class=\"term\" data-term-id=\"840\">download<\/span> since <span class=\"term\" data-term-id=\"319\">chronic<\/span> nasal infections<\/li>\n<li>Lack of breast development.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-tratamiento-para-la-displasia-ectodermica\"><\/span>What is the treatment for ectodermal dysplasia?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>There is no specific treatment for ectodermal dysplasia. Managing the condition is by treating the various symptoms. Patients often need to be treated by a team of doctors and dentists, rather than a single professional.<\/p>\n<ul>\n<li>Patients with no or no sweat gland function should live in colder climates or in air-conditioned places at home, school, and work. Cold water baths or sprays can be helpful in maintaining normal body temperature.<\/li>\n<li>Artificial tears can be used to prevent damage to the <span class=\"term\" data-term-id=\"1032\">cornea<\/span> in patients with defective production of tears. <span class=\"term\" data-term-id=\"1875\">Saline<\/span> Aerosols can also be helpful.<\/li>\n<li>Saline irrigation of the nose <span class=\"term\" data-term-id=\"189\">mucous membrane<\/span> can help eliminate <span class=\"term\" data-term-id=\"1182\">purulent<\/span> Debris and prevent infection.<\/li>\n<li>Early dental evaluation and intervention are essential.<\/li>\n<li>Surgical procedures such as repairing a cleft palate can decrease facial deformities and improve speech.<\/li>\n<li>Wigs can be used to improve the appearance of patients with little or no hair.<\/li>\n<\/ul>\n<p>Most people with ectodermal dysplasia can lead full and productive lives once they understand how to manage their condition. Special attention should be paid to children if they sweat and <span class=\"term\" data-term-id=\"190\">bugger<\/span> There are abnormalities in production. Recurrent high fevers can cause seizures and neurological problems.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>Que es ectod\u00e9rmico displasia? La displasia ectod\u00e9rmica es un gran grupo de trastornos hereditarios caracterizados por una primario defecto en cabello, dientes, u\u00f1as o funci\u00f3n de las gl\u00e1ndulas sudor\u00edparas, adem\u00e1s&#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":[],"class_list":["post-8019","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/8019","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=8019"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/8019\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=8019"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=8019"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=8019"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}