{"id":8122,"date":"2020-04-27T06:31:56","date_gmt":"2020-04-27T09:31:56","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/enfermedad-de-paget-extramamaria-de-la-piel\/"},"modified":"2020-04-27T14:40:06","modified_gmt":"2020-04-27T17:40:06","slug":"extramammary-paget-disease-of-the-skin","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/en\/wiki\/enfermedad-de-paget-extramamaria-de-la-piel\/","title":{"rendered":"Extramammary Paget&#039;s disease of the skin"},"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\/enfermedad-de-paget-extramamaria-de-la-piel\/#%C2%BFQue-es-la-enfermedad-de-Paget-extramamaria-de-la-piel\" >What is extramammary Paget&#039;s disease of the skin?<\/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\/enfermedad-de-paget-extramamaria-de-la-piel\/#%C2%BFQuien-contrae-la-enfermedad-de-Paget-extramamaria\" >Who gets extramammary Paget&#039;s disease?<\/a><\/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\/enfermedad-de-paget-extramamaria-de-la-piel\/#%C2%BFComo-se-clasifica-la-enfermedad-de-Paget-extramamaria\" >How is extramammary Paget&#039;s disease classified?<\/a><\/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\/enfermedad-de-paget-extramamaria-de-la-piel\/#%C2%BFCuales-son-las-caracteristicas-clinicas-de-la-enfermedad-de-Paget-extramamaria\" >What are the clinical features of extramammary Paget&#039;s 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\/enfermedad-de-paget-extramamaria-de-la-piel\/#Enfermedad-de-Paget-extramamaria\" >Extramammary Paget&#039;s disease<\/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\/enfermedad-de-paget-extramamaria-de-la-piel\/#%C2%BFQue-sitios-estan-afectados\" >What sites are affected?<\/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\/enfermedad-de-paget-extramamaria-de-la-piel\/#%C2%BFComo-se-diagnostica-la-enfermedad-de-Paget-extramamaria\" >How is extramammary Paget&#039;s disease diagnosed?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/enfermedad-de-paget-extramamaria-de-la-piel\/#%C2%BFCual-es-el-tratamiento-de-la-enfermedad-de-Paget-extramamaria\" >What is the treatment for extramammary Paget&#039;s disease?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/enfermedad-de-paget-extramamaria-de-la-piel\/#%C2%BFCual-es-el-pronostico-para-la-enfermedad-de-Paget-extramamaria\" >What is the prognosis for extramammary Paget&#039;s disease?<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-es-la-enfermedad-de-Paget-extramamaria-de-la-piel\"><\/span>What is extramammary Paget&#039;s disease of the skin?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Extramammary Paget&#039;s disease of the skin is a rare intraepithelial <span class=\"term\" data-term-id=\"30\">Cancer<\/span> characterized by a <span class=\"term\" data-term-id=\"319\">chronic<\/span> <span class=\"term\" data-term-id=\"491\">eczema<\/span>-I like it <span class=\"term\" data-term-id=\"255\">eruption<\/span> about <span class=\"term\" data-term-id=\"629\">armpits<\/span> or <span class=\"term\" data-term-id=\"825\">anogenital<\/span> male and female regions. Under the microscope, extramammary Paget&#039;s disease is very similar to the more common Paget&#039;s disease of the breast that occurs in the breast.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQuien-contrae-la-enfermedad-de-Paget-extramamaria\"><\/span>Who gets extramammary Paget&#039;s disease?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Extramammary Paget&#039;s disease occurs most frequently in the <span class=\"term\" data-term-id=\"1004\">vulva<\/span> of women between 50 and 60 years old. It can also affect the <span class=\"term\" data-term-id=\"572\">armpit<\/span> and the anogenital area in males of similar age. It has been observed to be more common in Asians than Europeans and very rare in African Americans.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-clasifica-la-enfermedad-de-Paget-extramamaria\"><\/span>How is extramammary Paget&#039;s disease classified?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Extramammary Paget disease of the skin has been classified into several subtypes.<\/p>\n<ul>\n<li>Type 1a <span class=\"term\" data-term-id=\"949\">primary<\/span> <span class=\"term\" data-term-id=\"47\">cutaneous<\/span> Extramammary Paget&#039;s disease arises from <span class=\"term\" data-term-id=\"12\">apocrine glands<\/span> within <span class=\"term\" data-term-id=\"85\">epidermis<\/span> (<span class=\"term\" data-term-id=\"863\">in the place<\/span>) or underlying skin <span class=\"term\" data-term-id=\"14\">appendices<\/span>.<\/li>\n<li>Primary cutaneous extramammary Paget&#039;s disease type 1b (15-25%) is associated with <span class=\"term\" data-term-id=\"612\">invader<\/span> Paget&#039;s disease or adenocarcinoma in situ.<\/li>\n<li>Type 2 extramammary Paget&#039;s disease arises from an underlying anal or rectal adenocarcinoma.<\/li>\n<li>Type 3 extramammary Paget&#039;s disease originates from adenocarcinoma of the bladder.<\/li>\n<\/ul>\n<p>Sometimes extramammary Paget&#039;s disease has been present for 10-15 years before evidence of cancer or <span class=\"term\" data-term-id=\"383\">metastasis<\/span> appears.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCuales-son-las-caracteristicas-clinicas-de-la-enfermedad-de-Paget-extramamaria\"><\/span>What are the clinical features of extramammary Paget&#039;s disease?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The most common <span class=\"term\" data-term-id=\"902\">symptom<\/span> of extramammary Paget&#039;s disease of the skin is mild to severe itching of a <span class=\"term\" data-term-id=\"164\">injury<\/span> found around the groin, genitals, <span class=\"term\" data-term-id=\"889\">perineum<\/span> or <span class=\"term\" data-term-id=\"1192\">perianal<\/span> zone. Pain and bleeding can occur from scratching injuries that have been around for a long time. Thickened <span class=\"term\" data-term-id=\"368\">plates<\/span> can form and turn red, <span class=\"term\" data-term-id=\"385\">scaly<\/span> and crispy <\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Enfermedad-de-Paget-extramamaria\"><\/span>Extramammary Paget&#039;s disease<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='extra-paget3__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisinkildfd-7017465-1129650' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/extra-paget3__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsInkiLDFd-7017465.jpg'>\n<\/div>\n<p class=\"p-small\">Armpit<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='extra-paget5__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisinkildg1xq-5600810-4289280' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/extra-paget5__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsInkiLDg1XQ-5600810.jpg'>\n<\/div>\n<p class=\"p-small\">Vulva<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='extra-paget-24__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-7579895-2882563' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/extra-paget-24__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-7579895.jpg'>\n<\/div>\n<p class=\"p-small\">Scrotum<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-sitios-estan-afectados\"><\/span>What sites are affected?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>In women, the most common area involved is the vulva. The first symptoms are usually itching and burning in one or more persistent plaques. These can spread to the labia, pubis, vagina, and thighs. Perianal lesions can extend to the anal canal.<\/p>\n<p>In men, the most common area is the scrotum followed by perianal disease, penis, and groin.<\/p>\n<p>The armpit is a rare site for extramammary Paget disease of the skin.<\/p>\n<p>The location of extramammary Paget&#039;s disease is useful in predicting the associated cancer risk. For example, 25\u201335% from extramammary Paget&#039;s disease arising near the anus is associated with underlying colorectal cancer.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-diagnostica-la-enfermedad-de-Paget-extramamaria\"><\/span>How is extramammary Paget&#039;s disease diagnosed?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Skin <span class=\"term\" data-term-id=\"24\">biopsy<\/span> of the lesion is done to get an accurate diagnosis of extramammary Paget&#039;s disease, as there are several other genital skin diseases that may appear similar. Below <span class=\"term\" data-term-id=\"867\">microscopy<\/span>, the presence of Paget cells along with other <span class=\"term\" data-term-id=\"474\">histological<\/span> The findings confirm the diagnosis. Special spots may be necessary to distinguish Paget&#039;s disease from early <span class=\"term\" data-term-id=\"1670\">melanoma<\/span> (melanoma in situ). See extramammary Paget&#039;s disease <span class=\"term\" data-term-id=\"223\">pathology<\/span>.<\/p>\n<p>Other tests may include:<\/p>\n<ul>\n<li>Assessment <span class=\"term\" data-term-id=\"1775\">lymph nodes<\/span> by <span class=\"term\" data-term-id=\"1378\">ultrasound<\/span> scan or fine needle <span class=\"term\" data-term-id=\"1869\">Aspire<\/span>\n<\/li>\n<li>Look for other malignancies, including Pap smear (cervical smear), pelvic imaging to detect underlying cancer, <span class=\"term\" data-term-id=\"1553\">colonoscopy<\/span>, mammogram<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-tratamiento-de-la-enfermedad-de-Paget-extramamaria\"><\/span>What is the treatment for extramammary Paget&#039;s disease?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Wide local <span class=\"term\" data-term-id=\"848\">excision<\/span>, vulvectomy or, if available, margin-controlled surgical excision (Mohs micrographic surgery) is the standard treatment for extramammary Paget disease. Paraffin sections are preferred over frozen sections. The margin is sometimes difficult to define when the lesions spread sporadically throughout the anogenital region. Reconstruction may require skin graft or flap repair.<\/p>\n<p><span class=\"term\" data-term-id=\"1074\">Reappearance<\/span> is common (30\u201350%), so patients should be reexamined every three months after surgery for the next two years, after which yearly follow-ups are recommended. Recurrence usually leads to further surgery.<\/p>\n<p>Non-surgical treatments for <span class=\"term\" data-term-id=\"805\">recurrent<\/span> the disease may include:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"1325\">Radiotherapy<\/span><\/li>\n<li>\n<span class=\"term\" data-term-id=\"1617\">To be<\/span> <span class=\"term\" data-term-id=\"1754\">ablation<\/span>\n<\/li>\n<li><span class=\"term\" data-term-id=\"1571\">Photodynamic<\/span> therapy<\/li>\n<li>5-fluorouracil <span class=\"term\" data-term-id=\"1121\">cream<\/span><\/li>\n<li>\n<p>Imiquimod cream.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-pronostico-para-la-enfermedad-de-Paget-extramamaria\"><\/span>What is the prognosis for extramammary Paget&#039;s disease?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Paget&#039;s disease has a low mortality. <span class=\"term\" data-term-id=\"241\">Forecast<\/span> it is worse if there is underlying cancer, the patient is over 70 years old, and Paget&#039;s disease is in the anal region.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>\u00bfQu\u00e9 es la enfermedad de Paget extramamaria de la piel? La enfermedad de Paget extramamaria de la piel es una intraepitelial rara c\u00e1ncer caracterizado por un cr\u00f3nico eczema-me gusta erupci\u00f3n&#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-8122","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/8122","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=8122"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/8122\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=8122"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=8122"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=8122"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}