{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T20:15:17Z","timestamp":1773173717351,"version":"3.50.1"},"reference-count":31,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,3,7]],"date-time":"2023-03-07T00:00:00Z","timestamp":1678147200000},"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":[[2023,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>DITRA, acronym for deficiency of interleukin\u201036 receptor antagonist (IL36RN), leads to unopposed pro\u2010inflammatory signalling which typically manifests as pustular psoriasis. In Asian patients, c.115\u2009+\u20096\u2009T\u2009&gt;\u2009C mutation is the most common and important single\u2010nucleotide variant in DITRA. We present the largest case series consisting of 58 DITRA patients carrying heterozygous or homozygous c.115\u2009+\u20096\u2009T\u2009&gt;\u2009C mutation. The mean age of onset (\u00b1SD) was 20.74 (\u00b120.86), and the median age of onset was 13\u2009years old. Twelve patients (20.7%) had disease onset before the age of two. Twenty\u2010two patients (37.9%) had disease onset between the ages of 2\u201318. Main clinical phenotype was generalized pustular psoriasis (GPP) with systemic symptoms (33 patients, 56.9%), followed by acrodermatitis continua of Hallopeau (ACH) (16 patients, 27.6%). Nearly half of our patients (27 patients, 46.6%) ever had ACH, and only three of them are free of ACH currently, which indicates that the development of ACH is relatively persistent and irreversible. Thirty\u2010four patients (58.6%) had recurrent GPP and 29 patients (50%) have been admitted due to GPP flare. Compared to those with heterozygous (C\/T) mutation, more patients carrying homozygous mutation (C\/C) have recurrent episodes of GPP (C\/T vs. C\/C: 25.53 vs. 76.47%, <jats:italic>p<\/jats:italic>\u00a0=\u00a00.0367). Two patients with squamous cell carcinomas arising from the pustular psoriasis skin lesions were noted. Two patients had elevated serum IgG4 levels.<\/jats:p>","DOI":"10.1111\/exd.14783","type":"journal-article","created":{"date-parts":[[2023,2,27]],"date-time":"2023-02-27T06:54:41Z","timestamp":1677480881000},"page":"1272-1278","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Deficiency of interleukin\u201036 receptor antagonist (<scp>DITRA<\/scp>): An analysis of 58 Chinese patients in a tertiary hospital in Taiwan"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9740-9720","authenticated-orcid":false,"given":"Chang\u2010Yu","family":"Hsieh","sequence":"first","affiliation":[{"name":"National Taiwan University Hospital Department of Dermatology  Taipei 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