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Recently, several case reports have demonstrated successful treatment of PRP with anti-IL7A and anti-IL12\/anti-IL23 monoclonal antibodies. We report a case of atypical juvenile PRP definitively diagnosed during adulthood with presence of CARD14 mutation. This case demonstrates a dramatic improvement with ustekinumab and highlights the role of genetic testing in chronic disease of diagnostic uncertainty.<\/jats:p>","DOI":"10.1002\/ski2.389","type":"journal-article","created":{"date-parts":[[2024,4,11]],"date-time":"2024-04-11T06:50:55Z","timestamp":1712818255000},"source":"Crossref","is-referenced-by-count":1,"title":["Severe Atypical Juvenile Pityriasis Rubra Pilaris Diagnosed in Adulthood with a Dramatic Improvement with Ustekinumab"],"prefix":"10.1093","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7126-8384","authenticated-orcid":false,"given":"Rebecca L.","family":"McCarthy","sequence":"first","affiliation":[{"name":"Faculty of Medicine and Dentistry, Centre for Cell Biology and Cutaneous Research, Blizard Institute, Queen Mary University of London , London ,","place":["UK"]},{"name":"Department of Dermatology, Royal London Hospital, Barts Health NHS Trust , London 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Dermatology, Royal London Hospital, Barts Health NHS Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edel A.","family":"O\u2019Toole","sequence":"additional","affiliation":[{"name":"Faculty of Medicine and Dentistry, Centre for Cell Biology and Cutaneous Research, Blizard Institute, Queen Mary University of London , London ,","place":["UK"]},{"name":"Department of Dermatology, Royal London Hospital, Barts Health NHS Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,4,11]]},"reference":[{"issue":"3","key":"2025112605421737700_ski2389-bib-0001","doi-asserted-by":"publisher","first-page":"377","DOI":"10.1007\/s40257-017-0338-1","article-title":"A review on pityriasis rubra pilaris","volume":"19","author":"Wang","year":"2018","journal-title":"Am J Clin 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