{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T13:17:37Z","timestamp":1773148657875,"version":"3.50.1"},"reference-count":17,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,4,17]],"date-time":"2020-04-17T00:00:00Z","timestamp":1587081600000},"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":["Pediatric Dermatology"],"published-print":{"date-parts":[[2020,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Generalized pustular psoriasis (GPP) is a severe form of psoriasis, which is rare in pediatric and adult patients. It is characterized by sterile pustular lesions that appear on erythematous skin, associated with systemic features. A recent identification of mutations in the <jats:italic>IL36RN<\/jats:italic> gene in some GPP patients has led to a diagnosis of new autoinflammatory disease, interleukin\u201036\u2010receptor antagonist deficiency (DITRA). DITRA represents an emerging group of autoinflammatory diseases with hyperkeratotic skin involvement, called autoinflammatory keratinization diseases (AIKD). DITRA diagnosis and management are challenging as neither DITRA\u2010specific clinical assessment tools nor treatment trials exist. Autoinflammatory Diseases Activity Index (AIDAI) is a validated tool originally developed to evaluate disease activity and treatment response in other inherited autoinflammatory diseases with systemic and skin involvement. We report the first use of AIDAI in a pediatric DITRA patient with the following goals: (a) to describe the contribution of AIDAI to our patient's management; (b) to identify potential limitations of AIDAI in DITRA; (c) to review literature for current psoriasis assessment tools; and (d) to propose a preliminary DITRA\/AIKD disease activity index (DITRA\/AIDAI) to be validated in future studies.<\/jats:p>","DOI":"10.1111\/pde.14155","type":"journal-article","created":{"date-parts":[[2020,4,17]],"date-time":"2020-04-17T07:03:51Z","timestamp":1587107031000},"page":"670-676","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Novel use of Autoinflammatory Diseases Activity Index (AIDAI) captures skin and extracutaneous features to help manage pediatric DITRA: A case report and a proposal for a modified disease activity index in autoinflammatory keratinization disorders"],"prefix":"10.1111","volume":"37","author":[{"given":"Chloe","family":"Stephenson","sequence":"first","affiliation":[{"name":"Department of Paediatrics University of Calgary Calgary AB Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vimal H.","family":"Prajapati","sequence":"additional","affiliation":[{"name":"Division of Dermatology Department of Medicine University of Calgary Calgary AB Canada"},{"name":"Division of Community Paediatrics Department of Paediatrics University of Calgary Calgary AB Canada"},{"name":"Division of Paediatric Rheumatology Department of Paediatrics University of Calgary Calgary AB Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charlene","family":"Hunter","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Calgary Calgary AB Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8769-269X","authenticated-orcid":false,"given":"Paivi","family":"Miettunen","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Calgary Calgary AB Canada"},{"name":"Division of Paediatric Rheumatology Department of Paediatrics University of Calgary Calgary AB Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,4,17]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijd.12525"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1013068"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2017.05.019"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2018.11.009"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.16104"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.annder.2019.03.007"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2013.138"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2011.01.022"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2004.031237"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2013.07.040"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jval.2013.07.002"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1159\/000250839"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2009.391"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.150126"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2013-203666"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-016-1750-y"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2011.07.022"}],"container-title":["Pediatric Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpde.14155","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.14155","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pde.14155","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.14155","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T08:25:07Z","timestamp":1693902307000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pde.14155"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,4,17]]},"references-count":17,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2020,7]]}},"alternative-id":["10.1111\/pde.14155"],"URL":"https:\/\/doi.org\/10.1111\/pde.14155","archive":["Portico"],"relation":{},"ISSN":["0736-8046","1525-1470"],"issn-type":[{"value":"0736-8046","type":"print"},{"value":"1525-1470","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,4,17]]},"assertion":[{"value":"2019-07-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-03-03","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-04-17","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}