{"id":11070,"date":"2023-09-19T13:28:00","date_gmt":"2023-09-19T11:28:00","guid":{"rendered":"https:\/\/www.adacyte.com\/?post_type=scientific-corner&#038;p=11070"},"modified":"2025-08-08T13:33:42","modified_gmt":"2025-08-08T11:33:42","slug":"cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature","status":"publish","type":"scientific-corner","link":"https:\/\/www.adacyte.com\/de\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/","title":{"rendered":"Cutaneous findings and treatments in deficiency of interleukin-36 receptor antagonist (DITRA): A review of the literature"},"content":{"rendered":"<div class=\"post-content max-w-987 mx-auto px-20 lg:px-0\">\n<p><a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/?sort=date&amp;term=Okorie+CL&amp;cauthor_id=37726967\">Chiamaka L Okorie<\/a><sup>\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/37726967\/#full-view-affiliation-1\">1<\/a><\/sup>,\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/?sort=date&amp;term=Nayudu+K&amp;cauthor_id=37726967\">Krithika Nayudu<\/a><sup>\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/37726967\/#full-view-affiliation-2\">2<\/a><\/sup>,\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/?sort=date&amp;term=Nambudiri+VE&amp;cauthor_id=37726967\">Vinod E Nambudiri<\/a><sup>\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/37726967\/#full-view-affiliation-3\">3<\/a>\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/37726967\/#full-view-affiliation-4\">4<\/a><\/sup><\/p>\n<\/div>\n\n<div class=\"post-content max-w-987 mx-auto px-20 lg:px-0\">\n<p>Exp Dermatol.\u00a02024 Jan;33(1):e14934.\u00a0doi: 10.1111\/exd.14934.\u00a0Epub 2023 Sep 19.<\/p>\n<\/div>\n\n<div class=\"post-content max-w-987 mx-auto px-20 lg:px-0\">\n<p><span style=\"color: rgb(33, 33, 33); font-family: BlinkMacSystemFont, -apple-system, &quot;Segoe UI&quot;, Roboto, Oxygen, Ubuntu, Cantarell, &quot;Fira Sans&quot;, &quot;Droid Sans&quot;, &quot;Helvetica Neue&quot;, sans-serif; font-size: 16px; white-space-collapse: collapse;\">Deficiency of the interleukin-36 receptor antagonist (DITRA) is a rare autoinflammatory disorder caused by mutations in the IL36RN gene. This mutation leads to a lack of functional interleukin-36 receptor antagonists (IL-36Ra), which results in an overactive immune system and chronic inflammation. Despite its rarity, numerous case series and individual reports in the literature emphasize the importance of recognizing and managing DITRA. Early identification of the cutaneous signs of DITRA is crucial for accurate diagnosis and timely administration of appropriate treatment. This review article provides a comprehensive overview of the current understanding of the cutaneous, non-cutaneous and histopathological manifestations of DITRA, with a focus on reported treatments. The disease typically presents in early childhood, although the age of onset can vary. Patients with DITRA exhibit recurrent episodes of skin inflammation, often with a pustular or pustular psoriasis-like appearance. Additionally, non-cutaneous manifestations are common, with recurrent fevers and elevated acute-phase reactants being the most prevalent. The exact prevalence of DITRA is unknown. Some cases of loss-of-function mutations in the IL36RN gene, considered a hallmark for diagnosis, have been identified in patients with familial generalized pustular psoriasis (GPP). Biological therapies with inhibition of IL-12\/23 and IL-17 are promising treatment options; paediatric patients with DITRA have shown complete response with mild relapses. New and emerging biologic therapeutics targeting the IL-36 pathway are also of interest in the management of this rare autoinflammatory disorder.<\/span><\/p>\n<\/div>\n\n<div class=\"post-content max-w-987 mx-auto px-20 lg:px-0\">\n<p>5.3 Granulocyte and monocyte apheresis (GMA) The use of GMA warrants further exploration.<\/p>\n<\/div>\n\n<div class=\"post-content max-w-987 mx-auto px-20 lg:px-0\">\n<p><\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"<p>Chiamaka L Okorie\u00a01,\u00a0Krithika Nayudu\u00a02,\u00a0Vinod E Nambudiri\u00a03\u00a04 Exp Dermatol.\u00a02024 Jan;33(1):e14934.\u00a0doi: 10.1111\/exd.14934.\u00a0Epub 2023 Sep 19. Deficiency of the interleukin-36 receptor antagonist (DITRA) is a rare autoinflammatory disorder caused by mutations in the IL36RN gene. This mutation leads to a lack of functional interleukin-36 receptor antagonists (IL-36Ra), which results in an overactive immune system and chronic inflammation. Despite [&hellip;]<\/p>\n","protected":false},"template":"","scientific_tags":[698,892,711,134,151,867,348,424,181,658,130,405,893],"class_list":["post-11070","scientific-corner","type-scientific-corner","status-publish","hentry","scientific_tags-anti-il-17a","scientific_tags-anti-il12-23","scientific_tags-autoinflammmatory","scientific_tags-biologics","scientific_tags-ditra","scientific_tags-extradermatological-manifestations","scientific_tags-generalized-pustular-psoriasis","scientific_tags-gma","scientific_tags-gmas-responders","scientific_tags-il-36","scientific_tags-il36rn","scientific_tags-pediatric","scientific_tags-rare"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v22.9 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Cutaneous findings and treatments in deficiency of interleukin-36 receptor antagonist (DITRA): A review of the literature - Adacyte<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/\" \/>\n<meta property=\"og:locale\" content=\"de_DE\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Cutaneous findings and treatments in deficiency of interleukin-36 receptor antagonist (DITRA): A review of the literature - Adacyte\" \/>\n<meta property=\"og:description\" content=\"Chiamaka L Okorie\u00a01,\u00a0Krithika Nayudu\u00a02,\u00a0Vinod E Nambudiri\u00a03\u00a04 Exp Dermatol.\u00a02024 Jan;33(1):e14934.\u00a0doi: 10.1111\/exd.14934.\u00a0Epub 2023 Sep 19. Deficiency of the interleukin-36 receptor antagonist (DITRA) is a rare autoinflammatory disorder caused by mutations in the IL36RN gene. This mutation leads to a lack of functional interleukin-36 receptor antagonists (IL-36Ra), which results in an overactive immune system and chronic inflammation. Despite [&hellip;]\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/\" \/>\n<meta property=\"og:site_name\" content=\"Adacyte\" \/>\n<meta property=\"article:modified_time\" content=\"2025-08-08T11:33:42+00:00\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Gesch\u00e4tzte Lesezeit\" \/>\n\t<meta name=\"twitter:data1\" content=\"2\u00a0Minuten\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/\",\"url\":\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/\",\"name\":\"Cutaneous findings and treatments in deficiency of interleukin-36 receptor antagonist (DITRA): A review of the literature - Adacyte\",\"isPartOf\":{\"@id\":\"https:\/\/www.adacyte.com\/#website\"},\"datePublished\":\"2023-09-19T11:28:00+00:00\",\"dateModified\":\"2025-08-08T11:33:42+00:00\",\"inLanguage\":\"de\",\"potentialAction\":[{\"@type\":\"ReadAction\",\"target\":[\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/\"]}]},{\"@type\":\"WebSite\",\"@id\":\"https:\/\/www.adacyte.com\/#website\",\"url\":\"https:\/\/www.adacyte.com\/\",\"name\":\"Adacyte\",\"description\":\"Adacyte Therapeutics\",\"publisher\":{\"@id\":\"https:\/\/www.adacyte.com\/#organization\"},\"potentialAction\":[{\"@type\":\"SearchAction\",\"target\":{\"@type\":\"EntryPoint\",\"urlTemplate\":\"https:\/\/www.adacyte.com\/?s={search_term_string}\"},\"query-input\":\"required name=search_term_string\"}],\"inLanguage\":\"de\"},{\"@type\":\"Organization\",\"@id\":\"https:\/\/www.adacyte.com\/#organization\",\"name\":\"Adacyte\",\"url\":\"https:\/\/www.adacyte.com\/\",\"logo\":{\"@type\":\"ImageObject\",\"inLanguage\":\"de\",\"@id\":\"https:\/\/www.adacyte.com\/#\/schema\/logo\/image\/\",\"url\":\"https:\/\/www.adacyte.com\/wp-content\/uploads\/2021\/01\/logo-adacyte.svg\",\"contentUrl\":\"https:\/\/www.adacyte.com\/wp-content\/uploads\/2021\/01\/logo-adacyte.svg\",\"width\":165,\"height\":46,\"caption\":\"Adacyte\"},\"image\":{\"@id\":\"https:\/\/www.adacyte.com\/#\/schema\/logo\/image\/\"}}]}<\/script>\n<!-- \/ Yoast SEO plugin. -->","yoast_head_json":{"title":"Cutaneous findings and treatments in deficiency of interleukin-36 receptor antagonist (DITRA): A review of the literature - Adacyte","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/","og_locale":"de_DE","og_type":"article","og_title":"Cutaneous findings and treatments in deficiency of interleukin-36 receptor antagonist (DITRA): A review of the literature - Adacyte","og_description":"Chiamaka L Okorie\u00a01,\u00a0Krithika Nayudu\u00a02,\u00a0Vinod E Nambudiri\u00a03\u00a04 Exp Dermatol.\u00a02024 Jan;33(1):e14934.\u00a0doi: 10.1111\/exd.14934.\u00a0Epub 2023 Sep 19. Deficiency of the interleukin-36 receptor antagonist (DITRA) is a rare autoinflammatory disorder caused by mutations in the IL36RN gene. This mutation leads to a lack of functional interleukin-36 receptor antagonists (IL-36Ra), which results in an overactive immune system and chronic inflammation. Despite [&hellip;]","og_url":"https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/","og_site_name":"Adacyte","article_modified_time":"2025-08-08T11:33:42+00:00","twitter_card":"summary_large_image","twitter_misc":{"Gesch\u00e4tzte Lesezeit":"2\u00a0Minuten"},"schema":{"@context":"https:\/\/schema.org","@graph":[{"@type":"WebPage","@id":"https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/","url":"https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/","name":"Cutaneous findings and treatments in deficiency of interleukin-36 receptor antagonist (DITRA): A review of the literature - Adacyte","isPartOf":{"@id":"https:\/\/www.adacyte.com\/#website"},"datePublished":"2023-09-19T11:28:00+00:00","dateModified":"2025-08-08T11:33:42+00:00","inLanguage":"de","potentialAction":[{"@type":"ReadAction","target":["https:\/\/www.adacyte.com\/professional\/scientific-corner\/cutaneous-findings-and-treatments-in-deficiency-of-interleukin-36-receptor-antagonist-ditra-a-review-of-the-literature\/"]}]},{"@type":"WebSite","@id":"https:\/\/www.adacyte.com\/#website","url":"https:\/\/www.adacyte.com\/","name":"Adacyte","description":"Adacyte Therapeutics","publisher":{"@id":"https:\/\/www.adacyte.com\/#organization"},"potentialAction":[{"@type":"SearchAction","target":{"@type":"EntryPoint","urlTemplate":"https:\/\/www.adacyte.com\/?s={search_term_string}"},"query-input":"required name=search_term_string"}],"inLanguage":"de"},{"@type":"Organization","@id":"https:\/\/www.adacyte.com\/#organization","name":"Adacyte","url":"https:\/\/www.adacyte.com\/","logo":{"@type":"ImageObject","inLanguage":"de","@id":"https:\/\/www.adacyte.com\/#\/schema\/logo\/image\/","url":"https:\/\/www.adacyte.com\/wp-content\/uploads\/2021\/01\/logo-adacyte.svg","contentUrl":"https:\/\/www.adacyte.com\/wp-content\/uploads\/2021\/01\/logo-adacyte.svg","width":165,"height":46,"caption":"Adacyte"},"image":{"@id":"https:\/\/www.adacyte.com\/#\/schema\/logo\/image\/"}}]}},"_links":{"self":[{"href":"https:\/\/www.adacyte.com\/de\/wp-json\/wp\/v2\/scientific-corner\/11070","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.adacyte.com\/de\/wp-json\/wp\/v2\/scientific-corner"}],"about":[{"href":"https:\/\/www.adacyte.com\/de\/wp-json\/wp\/v2\/types\/scientific-corner"}],"wp:attachment":[{"href":"https:\/\/www.adacyte.com\/de\/wp-json\/wp\/v2\/media?parent=11070"}],"wp:term":[{"taxonomy":"scientific_tags","embeddable":true,"href":"https:\/\/www.adacyte.com\/de\/wp-json\/wp\/v2\/scientific_tags?post=11070"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}