{"id":12186,"date":"2025-01-14T18:58:59","date_gmt":"2025-01-14T17:58:59","guid":{"rendered":"https:\/\/valintermed.com\/?p=12186"},"modified":"2025-01-14T18:58:59","modified_gmt":"2025-01-14T17:58:59","slug":"sindrom-mnozhestvennogo-krylovidnogo-lishaya-stseplennyy-s-h-hromosomoy","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/multiple-pterygoid-syndrome\/","title":{"rendered":"X-linked multiple pterygoid syndrome"},"content":{"rendered":"<div class=\"fpm_start\"><\/div>\n<p>X-linked multiple pterygoid syndrome (MPPS) is a rare genetic disorder characterized by abnormal development of the upper body and limbs. The condition is also associated with chromosomal structure, which explains its X-linkage. The main manifestations of the syndrome are multiple skin lesions in the form of lichen pterygoid, as well as abnormalities in the development of bones and soft tissues. The disease can lead to a variety of physical and psychosocial consequences in patients, since damage to the skin and limb structures can limit their physical activity and interaction with the outside world. At the same time, MPPS is associated with various comorbid conditions that require careful monitoring and medical intervention.<\/p>\n<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_85 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\/multiple-pterygoid-syndrome\/#%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\/multiple-pterygoid-syndrome\/#%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\/multiple-pterygoid-syndrome\/#%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\/multiple-pterygoid-syndrome\/#%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\/multiple-pterygoid-syndrome\/#%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\/multiple-pterygoid-syndrome\/#%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\/multiple-pterygoid-syndrome\/#%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\/multiple-pterygoid-syndrome\/#%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\/multiple-pterygoid-syndrome\/#%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\/multiple-pterygoid-syndrome\/#%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><\/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>Lichen pterygoideus multiplex syndrome was first described in the scientific literature in the 20th century; its uniqueness and complexity of pathogenesis attracted the attention of researchers. Key studies conducted in the 1970s and 1980s helped to establish the genetic nature of the disease and its association with the X chromosome. Interestingly, in the early 1990s, significant work was done to map the genes associated with the syndrome, which opened up new horizons for understanding the mechanism of its development. An important step in research was the use of molecular genetics and bioinformatics to analyze the mutations that cause the syndrome. Thus, the development of medical technologies contributed to a deeper understanding of the syndrome and an improvement in the quality of life of patients suffering from its manifestations.<\/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>The epidemiology of multiple pterygoid lichen syndrome demonstrates its rarity in the population. According to various studies, the incidence of this disease is approximately 1:100,000 - 1:200,000 live births. Women are less susceptible to this syndrome, but the disease is X-linked, which determines its prevalence in highly developing countries. One of the latest articles devoted to this aspect states that among patients with the syndrome, there is a high proportion of people with comorbidities such as hypertension and diabetes, which makes its study multifaceted and complex.<\/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>Genetic predisposition to multiple pterygoid lichen syndrome is directly related to mutations in genes located on the X chromosome. The most frequently mentioned gene is the gene responsible for the synthesis of certain proteins, a participant in metroabrasive processes in the skin epithelium. Mutations in this gene lead to disruption of the normal formation of skin and tissues. According to the latest data, more than 70% cases are associated with hereditary genetic changes transmitted through the maternal line. It is important to note that in particular in families where there have been cases of the disease, the likelihood of relapse increases significantly, which emphasizes the importance of genetic counseling for future parents.<\/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>Despite genetic causes, there are also external factors that can increase the risk of developing multiple pterygoid syndrome. The main risk factors include:<\/p>\n<ul>\n<li>Environmental exposures, including exposure to toxic chemicals, that may affect fetal development.<\/li>\n<li>Physical factors such as ionizing radiation, which can cause mutations in genes.<\/li>\n<li>Infectious diseases suffered by the mother during pregnancy, which can lead to developmental disorders in the fetus.<\/li>\n<li>Drug addiction or drug use by the mother during pregnancy, which can also negatively affect the genetic material.<\/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 multiple pterygoid syndrome involves several important steps. The main symptoms are multiple skin lesions located symmetrically on both sides of the body. Laboratory tests can help confirm the diagnosis, including genetic testing for mutations in specific genes. Radiological examinations, such as X-rays or MRIs, can be used to evaluate structural abnormalities in the bones and soft tissues. Various types of diagnostics include clinical examination, medical history, and individual genetic testing, which can exclude the possibility of other diseases with similar symptoms. Differential diagnosis is also essential to exclude dermatological pathologies, such as psoriasis or eczema, which can present with similar signs.<\/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 of multiple pterygoid syndrome should be individualized, taking into account the characteristics of each patient. The main approaches include:<\/p>\n<ul>\n<li>General treatment aimed at improving quality of life and reducing symptoms. This may include wound care, bandaging, and physical therapy.<\/li>\n<li>Pharmacological treatment: use of anti-inflammatory drugs, as well as agents to correct concomitant conditions, such as antibiotics for infections.<\/li>\n<li>Surgical treatment may be required in cases where anomalies lead to significant functional impairment, such as malformation of limbs.<\/li>\n<li>Other treatment methods may include psychological support and rehabilitation, which is very important for the psychological comfort of patients.<\/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 that may be used to treat multiple pterygoid syndrome include:<\/p>\n<ul>\n<li>Anti-inflammatory drugs: ibuprofen, naproxen.<\/li>\n<li>Antibiotics for the treatment of secondary infections: amoxicillin, cephalosporins.<\/li>\n<li>Immunomodulatory drugs: methotrexate.<\/li>\n<li>Physiotherapeutic agents: various external creams and ointments to improve the condition of the skin.<\/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 a patient with multiple pterygoid syndrome includes regular specialist consultations and quality of life assessment. Control stages may be performed every 3-6 months depending on the severity of the disease. The prognosis may vary depending on the severity of the syndrome and the presence of comorbidities, but early involvement and a comprehensive approach to treatment can significantly improve the outcome. Potential complications include secondary skin infections, limitations in movement, psychological problems such as depression and isolation.<\/p><script data-noptimize=\"\" data-wpfc-render=\"false\">\nfpm_start( \"true\" );\n<\/script>\n\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>Multiple pterygoid syndrome may present differently in different age groups. Newborns and younger children often have more severe symptoms due to their growth and development. Adolescence and adulthood may develop complications such as problems with self-esteem and integration into society. Adult patients are more likely to require surgery and long-term monitoring due to the increased risk of comorbidities.<\/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 are the main symptoms of multiple pterygoid syndrome?<\/strong> The main symptoms include multiple skin rashes, limb abnormalities and possible associated disorders.<\/li>\n<li><strong>Can multiple pterygoid syndrome be completely cured?<\/strong> A complete cure is not possible, but treatment can significantly improve the quality of life of patients.<\/li>\n<li><strong>Who is most susceptible to this disease?<\/strong> The disease is more common in men because it is X-linked. However, women can also be carriers.<\/li>\n<li><strong>What diagnostic methods are used now?<\/strong> Laboratory tests, genetic testing, X-rays and MRIs are used.<\/li>\n<li><strong>How can the quality of life of patients with this syndrome be improved?<\/strong> A comprehensive approach, including medical, psychological support and rehabilitation, significantly helps patients adapt to life with the syndrome.<\/li>\n<\/ul>\n<div class=\"fpm_end\"><\/div>","protected":false},"excerpt":{"rendered":"<p>X-linked multiple pterygoid syndrome (X-linked lichen multiplex syndrome) is a rare genetic disorder characterized by abnormal development of the upper body<\/p>","protected":false},"author":1,"featured_media":20948,"comment_status":"open","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[298],"tags":[],"class_list":["post-12186","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-medlibrary"],"_links":{"self":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12186","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=12186"}],"version-history":[{"count":1,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12186\/revisions"}],"predecessor-version":[{"id":14924,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12186\/revisions\/14924"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media\/20948"}],"wp:attachment":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media?parent=12186"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/categories?post=12186"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/tags?post=12186"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}