{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T11:18:23Z","timestamp":1788347903946,"version":"build-2803163510"},"reference-count":13,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T00:00:00Z","timestamp":1788307200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T00:00:00Z","timestamp":1788307200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Dermatology"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Papillon\u2013Lef\u00e8vre syndrome (PLS) is a rare autosomal recessive genodermatosis characterized by palmoplantar hyperkeratosis and severe periodontitis, leading to premature loss of both primary and permanent teeth, and treatment responses are often unsatisfactory. We report a 20\u2010year\u2010old female patient with PLS who presented with extensive palmoplantar hyperkeratosis and widespread psoriasiform plaques refractory to multiple conventional therapies. Treatment with ustekinumab resulted in marked clinical improvement by the sixth month after three subcutaneous injections. The patient has remained in sustained clinical remission during 6\u2009years of continuous therapy, with only mild and intermittent recurrent plaques on the knees, suggesting that biologic therapy may represent a sustainable option in selected cases of PLS.<\/jats:p>","DOI":"10.1111\/pde.70351","type":"journal-article","created":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T11:02:51Z","timestamp":1788346971000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Ustekinumab as an Effective Therapeutic Option in Papillon\u2013Lef\u00e8vre Syndrome: Long\u2010Term Follow\u2010Up"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6980-8986","authenticated-orcid":false,"given":"Ozlem Apti","family":"Sengkioun","sequence":"first","affiliation":[{"name":"Department of Dermatology Marmara University School of Medicine, Pendik Research and Training Hospital  Istanbul Turkey"},{"name":"Department of Dermatology Ac\u0131badem Mehmet Ali Ayd\u0131nlar University School of Medicine, Ac\u0131badem Altunizade Hospital  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4251-0581","authenticated-orcid":false,"given":"Deniz","family":"Y\u00fccelten","sequence":"additional","affiliation":[{"name":"Department of Dermatology Marmara University School of Medicine, Pendik Research and Training Hospital  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9935-6298","authenticated-orcid":false,"given":"T\u00fclin","family":"Ergun","sequence":"additional","affiliation":[{"name":"Department of Dermatology Marmara University School of Medicine, Pendik Research and Training Hospital  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,2]]},"reference":[{"issue":"6","key":"e_1_2_7_2_1","article-title":"Papillon\u2013Lef\u00e8vre Syndrome: Clinical, Immunologic, and Genetic Characteristics","volume":"32","author":"Al\u2010Shamrani S. M.","year":"2019","journal-title":"Dermatologic Therapy"},{"key":"e_1_2_7_3_1","article-title":"Papillon\u2013Lef\u00e8vre Syndrome: Updated Review of Clinical Features, Genetics, and Management","volume":"16","author":"Pham T. T.","year":"2021","journal-title":"Orphanet Journal of Rare Diseases"},{"key":"e_1_2_7_4_1","first-page":"81","article-title":"Papillon\u2013Lef\u00e8vre syndrome: a comprehensive review","volume":"13","author":"Dababneh R.","year":"2020","journal-title":"Clinical, Cosmetic and Investigational Dermatology"},{"issue":"9","key":"e_1_2_7_5_1","first-page":"1854","article-title":"CTSC Mutations and Genotype\u2013Phenotype Correlations in Papillon\u2013Lef\u00e8vre Syndrome","volume":"140","author":"Zhang Y.","year":"2020","journal-title":"Journal of Investigative Dermatology"},{"issue":"2","key":"e_1_2_7_6_1","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1136\/jmg.37.2.88","article-title":"Mutations of the Cathepsin C Gene Are Responsible for Papillon\u2013Lef\u00e8vre Syndrome","volume":"37","author":"Hart T. C.","year":"2000","journal-title":"Journal of Medical Genetics"},{"issue":"6","key":"e_1_2_7_7_1","doi-asserted-by":"crossref","first-page":"e798","DOI":"10.1111\/jdv.18938","article-title":"Novel Compound Heterozygous Mutation in CTSC Gene With Response to Ustekinumab","volume":"37","author":"Latour\u2010\u00c1lvarez I.","year":"2023","journal-title":"Journal of the European Academy of Dermatology and Venereology"},{"key":"e_1_2_7_8_1","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.jdcr.2022.11.003","article-title":"Dimethyl Fumarate for Treating Papillon\u2013Lef\u00e8vre Syndrome","volume":"31","author":"Al\u2010Omair A.","year":"2022","journal-title":"JAAD Case Reports"},{"issue":"3","key":"e_1_2_7_9_1","doi-asserted-by":"crossref","first-page":"e235","DOI":"10.1111\/jdv.17772","article-title":"Papillon\u2013Lef\u00e8vre Syndrome Treated by Acitretin: Cytokine Profile and Therapeutic Implications","volume":"36","author":"Leuenberger M.","year":"2022","journal-title":"Journal of the European Academy of Dermatology and Venereology"},{"issue":"8","key":"e_1_2_7_10_1","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1001\/jamadermatol.2025.1639","article-title":"Secukinumab and Upadacitinib Treatment for Papillon\u2013Lef\u00e8vre Syndrome in Two Cases","volume":"161","author":"Li J.","year":"2025","journal-title":"JAMA Dermatology"},{"issue":"1","key":"e_1_2_7_11_1","first-page":"e1","article-title":"Biologic and Targeted Therapies for Inherited Disorders of Keratinisation: A Systematic Review","volume":"65","author":"Chen M. K. Y.","year":"2024","journal-title":"Australasian Journal of Dermatology"},{"issue":"3","key":"e_1_2_7_12_1","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1111\/j.1600-051X.1996.tb02073.x","article-title":"Systemic Retinoid Medication and Periodontal Health in Patients With Papillon\u2013Lef\u00e8vre Syndrome","volume":"23","author":"Lundgren T.","year":"1996","journal-title":"Journal of Clinical Periodontology"},{"issue":"7","key":"e_1_2_7_13_1","doi-asserted-by":"crossref","first-page":"764","DOI":"10.1111\/apt.16802","article-title":"Systematic Review With Meta\u2010Analysis: Loss of Response and Requirement of Ustekinumab Dose Escalation in Inflammatory Bowel Diseases","volume":"55","author":"Yang H.","year":"2022","journal-title":"Alimentary Pharmacology & Therapeutics"},{"issue":"6","key":"e_1_2_7_14_1","article-title":"Ustekinumab Drug Survival in Patients With Psoriasis: A Real\u2010World Analysis","volume":"33","author":"Osuna C. G.","year":"2020","journal-title":"Dermatologic Therapy"}],"container-title":["Pediatric Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.70351","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pde.70351","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.70351","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T11:02:55Z","timestamp":1788346975000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pde.70351"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,2]]},"references-count":13,"alternative-id":["10.1111\/pde.70351"],"URL":"https:\/\/doi.org\/10.1111\/pde.70351","archive":["Portico"],"relation":{},"ISSN":["0736-8046","1525-1470"],"issn-type":[{"value":"0736-8046","type":"print"},{"value":"1525-1470","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,2]]},"assertion":[{"value":"2026-02-24","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-24","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-02","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"pde.70351"}}