{"id":11091,"date":"2025-07-27T12:26:38","date_gmt":"2025-07-27T10:26:38","guid":{"rendered":"https:\/\/valintermed.com\/?p=11091"},"modified":"2025-07-27T12:26:38","modified_gmt":"2025-07-27T10:26:38","slug":"ekstramammarnaya-bolezn-pedzheta","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/","title":{"rendered":"Extramammary Paget&#039;s disease"},"content":{"rendered":"<div class=\"fpm_start\"><\/div>\n<p>Extramammary Paget&#039;s disease is a rare malignant neoplasm characterized by changes in the skin and underlying tissues that are outside the nipples of the mammary gland. The disease is usually associated with invasive breast cancer, but unlike classic Paget&#039;s disease, its manifestations can develop in various areas of the body, including the genitals, anal area, and other areas of the skin. The extramammary form has its own diagnostic and treatment features, which requires attention from medical specialists. <\/p>\n<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_88 counter-flat ez-toc-counter ez-toc-light-blue ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Content<\/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\" 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href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\" >History of the disease and interesting historical facts<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F\" >Epidemiology<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E\" >Genetic predisposition to this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Risk factors for the development of this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Diagnosis of this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\" >Treatment<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >List of medications used to treat this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Disease monitoring<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Age-related features of the disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-10\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\" >Questions and Answers<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-11\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/extramammary-pedgetal-disease\/#%D0%A1%D0%BE%D0%B2%D0%B5%D1%82%D1%8B_%D0%BE%D1%82_%D0%B4%D0%BE%D0%BA%D1%82%D0%BE%D1%80%D0%B0_%D0%9E%D0%BB%D0%B5%D0%B3%D0%B0_%D0%9A%D0%BE%D1%80%D0%B6%D0%B8%D0%BA%D0%BE%D0%B2%D0%B0_%D0%BF%D0%BE_%D1%8D%D1%82%D0%BE%D0%B9_%D0%B1%D0%BE%D0%BB%D0%B5%D0%B7%D0%BD%D0%B8\" >Advice from Dr. Oleg Korzhikov on this disease<\/a><\/li><\/ul><\/nav><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\"><\/span>History of the disease and interesting historical facts<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Extramammary Paget&#039;s disease was first described in the late 19th century. It was named after the French physician Edmond Paget, who in 1874 reported his observations of skin lesions in the nipple area associated with carcinoma. In the following years, cases of the extramammary form, known as &quot;extramammary Paget&#039;s disease&quot;, were reported. Research continued for decades, but it was not until the 20th century that the disease was found to have specific clinical and pathogenetic characteristics. For example, in 2005, a number of studies were conducted that allowed a better understanding of the molecular biology of this pathology and its relationship with other malignancies.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F\"><\/span>Epidemiology<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Extramammary Paget&#039;s disease is a rare disease. Statistics show that there are only 1-3 new cases per 100,000 people per year. The gender ratio shows a higher incidence in women than in men, but cases in men have also been recorded. According to studies, most patients are elderly, and the average age at diagnosis is about 60-70 years. <\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E\"><\/span>Genetic predisposition to this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Although the genetic predisposition to extramammary Paget&#039;s disease remains unknown, research suggests that certain genes may play a role in the development of the disease. In particular, changes in the TP53 and HER2 genes have been associated with an increased risk of malignancies, including Paget&#039;s disease. Genetic mutations such as BRCA1 and BRCA2 may also increase the likelihood of developing cancers in women and, to some extent, in men, highlighting the importance of genetic screening for susceptible populations.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Risk factors for the development of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Risk factors that contribute to the development of extramammary Paget&#039;s disease include:<\/p>\n<ul>\n<li>Age - the risk increases with age.<\/li>\n<li>Genetic predisposition - having a family history of cancer.<\/li>\n<li>Smoking can contribute to the pathogenesis of various malignant tumors.<\/li>\n<li>Chemical exposure - long-term exposure to carcinogens.<\/li>\n<li>Human papillomavirus infection - some strains are associated with the development of skin cancer.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Diagnosis of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Diagnosis of extramammary Paget&#039;s disease requires careful consideration because symptoms may resemble other skin conditions. Key symptoms include:<\/p>\n<ul>\n<li>Redness and itching in the affected area.<\/li>\n<li>Peeling and erosion of the skin.<\/li>\n<li>Compaction of underlying tissues.<\/li>\n<\/ul>\n<p>Laboratory tests, such as biopsy, are needed to confirm the diagnosis. Radiological examinations, including ultrasound and MRI, can be used to assess the extent of the disease. It is important to differentiate the disease from other forms of skin disease, including eczema and psoriasis.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\"><\/span>Treatment<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Treatment for extramammary Paget&#039;s disease depends on the stage of the disease and may include:<\/p>\n<ul>\n<li>General treatment - aimed at improving the quality of life and symptomatic treatment.<\/li>\n<li>Pharmacological treatment - includes antitumor drugs such as taxol and anthracyclines.<\/li>\n<li>Surgical treatment - may include resection of the affected area of skin.<\/li>\n<li>Other treatments, such as immunotherapy and radiotherapy, may be used depending on clinical findings.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>List of medications used to treat this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Medications recommended for the treatment of extramammary Paget&#039;s disease include:<\/p><script data-noptimize=\"\" data-wpfc-render=\"false\">\nfpm_start( \"true\" );\n<\/script>\n\n<ul>\n<li>Cisplatin<\/li>\n<li>Doxorubicin<\/li>\n<li>Paclitaxel<\/li>\n<li>Imitozan<\/li>\n<li>Fluorouracil<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Disease monitoring<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Monitoring of patients with extramammary Paget&#039;s disease includes regular examinations, clinical assessments, and laboratory monitoring. The recurrence rate is approximately 30%, highlighting the importance of long-term follow-up. Prognosis depends on the stage at presentation and the presence of metastases. Potential complications may include recurrence and lymph node metastases.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Age-related features of the disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Different age groups exhibit different disease patterns. Older patients may have more aggressive disease, while younger patients may have less severe symptoms. Patients over 70 years of age are also more likely to have underlying medical conditions, which can complicate diagnosis and treatment.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\"><\/span>Questions and Answers<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<ul>\n<li><strong>What is extramammary Paget&#039;s disease?<\/strong><br \/>\n    Extramammary Paget&#039;s disease is a skin malignancy associated with cancer located outside the mammary glands.\n  <\/li>\n<li><strong>What risk factors may contribute to the development of the disease?<\/strong><br \/>\n    Risk factors include age, genetic predisposition, smoking and exposure to carcinogens.\n  <\/li>\n<li><strong>How is the disease diagnosed?<\/strong><br \/>\n    Diagnosis includes examination, skin biopsy, and radiological methods such as ultrasound and MRI.\n  <\/li>\n<li><strong>What treatment is used for this disease?<\/strong><br \/>\n    Treatment may be surgical, pharmacological, and include immunotherapy.\n  <\/li>\n<li><strong>What is the prognosis for this disease?<\/strong><br \/>\n    Prognosis depends on the stage of the disease and the presence of metastases; recurrent cases are observed in 30% patients.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A1%D0%BE%D0%B2%D0%B5%D1%82%D1%8B_%D0%BE%D1%82_%D0%B4%D0%BE%D0%BA%D1%82%D0%BE%D1%80%D0%B0_%D0%9E%D0%BB%D0%B5%D0%B3%D0%B0_%D0%9A%D0%BE%D1%80%D0%B6%D0%B8%D0%BA%D0%BE%D0%B2%D0%B0_%D0%BF%D0%BE_%D1%8D%D1%82%D0%BE%D0%B9_%D0%B1%D0%BE%D0%BB%D0%B5%D0%B7%D0%BD%D0%B8\"><\/span>Advice from Dr. Oleg Korzhikov on this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Dr. Oleg Korzhikov recommends that patients with extramammary Paget&#039;s disease not ignore any changes in the skin condition and be sure to undergo regular examinations. &quot;The disease indicators can be very changeable, and rapid intervention provides the best prognosis,&quot; he emphasizes. The doctor also advises maintaining a healthy lifestyle, including proper nutrition and physical activity, which can help maintain overall health and prevent relapses.<\/p>\n<div class=\"fpm_end\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Extramammary Paget&#039;s disease is a rare malignancy characterized by changes in the skin and underlying tissues outside the nipples of the mammary gland.<\/p>","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[298],"tags":[],"class_list":["post-11091","post","type-post","status-publish","format-standard","hentry","category-medlibrary"],"_links":{"self":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/11091","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/comments?post=11091"}],"version-history":[{"count":1,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/11091\/revisions"}],"predecessor-version":[{"id":16124,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/11091\/revisions\/16124"}],"wp:attachment":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media?parent=11091"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/categories?post=11091"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/tags?post=11091"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}