{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T23:50:34Z","timestamp":1793663434629,"version":"4.5.1"},"reference-count":39,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,9,19]],"date-time":"2023-09-19T00:00:00Z","timestamp":1695081600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Experimental Dermatology"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Deficiency of the interleukin\u201036 receptor antagonist (DITRA) is a rare autoinflammatory disorder caused by mutations in the\n                    <jats:italic>IL36RN<\/jats:italic>\n                    gene. This mutation leads to a lack of functional interleukin\u201036 receptor antagonists (IL\u201036Ra), 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\u2010cutaneous 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\u2010like appearance. Additionally, non\u2010cutaneous manifestations are common, with recurrent fevers and elevated acute\u2010phase reactants being the most prevalent. The exact prevalence of DITRA is unknown. Some cases of loss\u2010of\u2010function mutations in the\n                    <jats:italic>IL36RN<\/jats:italic>\n                    gene, considered a hallmark for diagnosis, have been identified in patients with familial generalized pustular psoriasis (GPP). Biological therapies with inhibition of IL\u201012\/23 and IL\u201017 are promising treatment options; paediatric patients with DITRA have shown complete response with mild relapses. New and emerging biologic therapeutics targeting the IL\u201036 pathway are also of interest in the management of this rare autoinflammatory disorder.\n                  <\/jats:p>","DOI":"10.1111\/exd.14934","type":"journal-article","created":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T01:32:48Z","timestamp":1695173568000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Cutaneous findings and treatments in deficiency of interleukin\u201036 receptor antagonist (\n                    <scp>DITRA<\/scp>\n                    ): A review of the literature"],"prefix":"10.1111","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2233-4557","authenticated-orcid":false,"given":"Chiamaka L.","family":"Okorie","sequence":"first","affiliation":[{"name":"Geisel School of Medicine at Dartmouth  Hanover New Hampshire 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