{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,29]],"date-time":"2025-06-29T04:41:27Z","timestamp":1751172087591,"version":"3.37.3"},"reference-count":62,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2022,11,11]],"date-time":"2022-11-11T00:00:00Z","timestamp":1668124800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Niigata Medical Association"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,3,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Epidermolytic ichthyosis (EI) is a major form of nonsyndromic inherited ichthyosis, characterized by erythroderma, marked hyperkeratosis and scale, bulla and erosion at birth, associated with KRT1\/KRT10 mutations. The cytokine and chemokine profiles in EI are poorly understood, and specific treatment options have not been established.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Aim<\/jats:title>\n                  <jats:p>To explore novel biomarkers and therapeutic targets in patients with EI.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We analysed cytokine levels in serum and skin samples from 10 patients with inherited ichthyosis, including seven patients with EI. Wild-type and mutant KRT1 constructs were established and transfected into HaCaT cells, an immortalized keratinocyte cell line, for in vitro immunoblotting and immunocytochemistry analyses.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Multiplex cytokine\/chemokine analysis revealed that 10 cytokines\/chemokines [interleukin (IL)-1\u03b2, IL-4, IL-17A, IL-16, IL-18, IL-1 receptor-\u03b1, macrophage colony-stimulating factor, interferon-\u03b12, basic fibroblast growth factor and monocyte chemotactic protein-3] were significantly increased in patients with EI. Furthermore, IL-18 levels were significantly higher in patients with EI [n = 7; 2714.1 (1438.0)\u2005pg\u2005mL\u22121] than in healthy controls [n = 11; 218.4 (28.4)\u2005pg\u2005mL\u22121, P &amp;lt; 0.01]. Immunohistochemical analyses showed that IL-18 expression was elevated in skin samples from patients with EI. Serum IL-18 levels correlated with the severity of ichthyosis, as measured by the Ichthyosis Scoring System. Immunoblotting analysis revealed that mature IL-18 levels were increased in the supernatant of mutant KRT1 expressing HaCaT cells. Additionally, these cells showed NLRP3 aggregation in the cytoplasm and ASC clustered around mutant keratin aggregations. These findings suggest that mutant keratin might promote the activation of the NLRP3 inflammasome and its downstream caspase-1-mediated IL-18 release in keratinocytes from patients with EI.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Our results suggest that serum IL-18 is a severity marker released from the skin of patients with EI. Blockade of IL-18 may be a useful novel therapeutic option for patients with EI.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ced\/llac069","type":"journal-article","created":{"date-parts":[[2023,1,19]],"date-time":"2023-01-19T14:26:00Z","timestamp":1674138360000},"page":"199-210","source":"Crossref","is-referenced-by-count":6,"title":["Interleukin-18 as a severity marker and novel potential therapeutic target for epidermolytic ichthyosis"],"prefix":"10.1093","volume":"48","author":[{"given":"Osamu","family":"Ansai","sequence":"first","affiliation":[{"name":"Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshinari","family":"Miyauchi","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryota","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsuya","family":"Katsumi","sequence":"additional","affiliation":[{"name":"Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomoki","family":"Nishiguchi","sequence":"additional","affiliation":[{"name":"Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akito","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoru","family":"Shinkuma","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Nara Medical University , Kashihara , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3865-6366","authenticated-orcid":false,"given":"Ken","family":"Natsuga","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshifumi","family":"Nomura","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Faculty of Medicine, University of Tsukuba , Tsukuba , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yutaka","family":"Shimomura","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Yamaguchi University Graduate School of Medicine , Ube , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Riichiro","family":"Abe","sequence":"additional","affiliation":[{"name":"Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2022,11,11]]},"reference":[{"key":"2023100310340270200_llac069-B1","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1016\/j.jaad.2009.11.020","article-title":"Revised nomenclature and classification of inherited ichthyoses: results of the First Ichthyosis Consensus Conference in Sor\u00e8ze 2009","volume":"63","author":"Oji","year":"2010","journal-title":"J Am Acad Dermatol"},{"key":"2023100310340270200_llac069-B2","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1111\/1346-8138.13243","article-title":"Inherited ichthyosis: non-syndromic forms","volume":"43","author":"Takeichi","year":"2016","journal-title":"J Dermatol"},{"key":"2023100310340270200_llac069-B3","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/S0955-0674(01)00301-5","article-title":"\u2018Hard\u2019 and \u2018soft\u2019 principles defining the structure, function and regulation of keratin intermediate filaments","volume":"14","author":"Coulombe","year":"2002","journal-title":"Curr Opin Cell Biol"},{"key":"2023100310340270200_llac069-B4","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1007\/s00418-008-0435-6","article-title":"The human keratins: biology and pathology","volume":"129","author":"Moll","year":"2008","journal-title":"Histochem Cell Biol"},{"key":"2023100310340270200_llac069-B5","doi-asserted-by":"crossref","first-page":"19537","DOI":"10.1073\/pnas.1309576110","article-title":"Keratin 16 regulates innate immunity in response to epidermal barrier breach","volume":"110","author":"Lessard","year":"2013","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2023100310340270200_llac069-B6","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1083\/jcb.201408026","article-title":"Regulation of C-X-C chemokine gene expression by keratin 17 and hnRNP K in skin tumor keratinocytes","volume":"208","author":"Chung","year":"2015","journal-title":"J Cell Biol"},{"key":"2023100310340270200_llac069-B7","doi-asserted-by":"crossref","first-page":"1461","DOI":"10.1016\/j.jaci.2016.04.046","article-title":"Keratin-dependent thymic stromal lymphopoietin expression suggests a link between skin blistering and atopic disease","volume":"138","author":"Kumar","year":"2016","journal-title":"J Allergy Clin Immunol"},{"key":"2023100310340270200_llac069-B8","first-page":"5269","article-title":"Keratin 1 maintains skin integrity and participates in an inflammatory network in skin via interleukin-18","volume":"125","author":"Roth","year":"2012","journal-title":"J Cell Sci"},{"key":"2023100310340270200_llac069-B9","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1046\/j.0309-2402.2003.02820.x","article-title":"Quality of life in adults with congenital ichthyosis","volume":"44","author":"G\u00e5nemo","year":"2003","journal-title":"J Adv Nurs"},{"key":"2023100310340270200_llac069-B10","first-page":"61","article-title":"Health-related quality of life among patients with ichthyosis","volume":"14","author":"G\u00e5nemo","year":"2004","journal-title":"Eur J Dermatol"},{"key":"2023100310340270200_llac069-B11","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1111\/j.1365-2133.2011.10701.x","article-title":"Factors influencing quality of life in patients with inherited ichthyosis: a qualitative study in adults using focus groups","volume":"166","author":"Mazereeuw-Hautier","year":"2012","journal-title":"Br J Dermatol"},{"key":"2023100310340270200_llac069-B12","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1111\/j.1525-1470.2010.01160.x","article-title":"Annual direct and indirect health costs of the congenital ichthyoses","volume":"27","author":"Styperek","year":"2010","journal-title":"Pediatr Dermatol"},{"key":"2023100310340270200_llac069-B13","doi-asserted-by":"crossref","first-page":"S28","DOI":"10.1016\/j.jaad.2017.12.022","article-title":"Dupilumab: a review of its use in the treatment of atopic dermatitis","volume":"78","author":"Gooderham","year":"2018","journal-title":"J Am Acad Dermatol"},{"key":"2023100310340270200_llac069-B14","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1111\/bjd.19039","article-title":"British Association of Dermatologists guidelines for biologic therapy for psoriasis 2020: a rapid update","volume":"183","author":"Smith","year":"2020","journal-title":"Br J Dermatol"},{"key":"2023100310340270200_llac069-B15","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1016\/j.jaci.2016.07.019","article-title":"An IL-17\u2013dominant immune profile is shared across the major orphan forms of ichthyosis","volume":"139","author":"Paller","year":"2017","journal-title":"J Allergy Clin Immunol"},{"key":"2023100310340270200_llac069-B16","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1016\/j.jaci.2018.03.021","article-title":"Ichthyosis molecular fingerprinting shows profound TH17 skewing and a unique barrier genomic signature","volume":"143","author":"Malik","year":"2019","journal-title":"J Allergy Clin Immunol"},{"key":"2023100310340270200_llac069-B17","doi-asserted-by":"publisher","DOI":"10.1007\/s00403-022-02325-3","article-title":"Secukinumab responses vary across the spectrum of congenital ichthyosis in adults","author":"Lefferdink","year":"2022","journal-title":"Arch Dermatol Res"},{"key":"2023100310340270200_llac069-B18","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1001\/jamadermatol.2021.5917","article-title":"Development and initial validation of a novel system to assess ichthyosis severity","volume":"158","author":"Sun","year":"2022","journal-title":"JAMA Dermatol"},{"key":"2023100310340270200_llac069-B19","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1002\/cia2.12230","article-title":"New insight of itch mediators and proinflammatory cytokines in epidermolysis bullosa","volume":"5","author":"Nguyen","year":"2022","journal-title":"J Cutan Immunol Allergy"},{"key":"2023100310340270200_llac069-B20","doi-asserted-by":"crossref","DOI":"10.1007\/978-3-319-24277-4","volume-title":"ggplot2: Elegant Graphics for Data Analysis","author":"Wickham","year":"2016"},{"key":"2023100310340270200_llac069-B21","doi-asserted-by":"crossref","first-page":"11340","DOI":"10.1073\/pnas.152337799","article-title":"IL-18 contributes to the spontaneous development of atopic dermatitis-like inflammatory skin lesion independently of IgE\/stat6 under specific pathogen-free conditions","volume":"99","author":"Konishi","year":"2002","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2023100310340270200_llac069-B22","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1111\/j.1365-2230.2011.04113.x","article-title":"Relationship between serum levels of interleukin-18, IgE and disease severity in patients with atopic dermatitis","volume":"36","author":"Trzeciak","year":"2011","journal-title":"Clin Exp Dermatol"},{"key":"2023100310340270200_llac069-B23","doi-asserted-by":"crossref","first-page":"1225","DOI":"10.1111\/j.0022-202X.2005.23715.x","article-title":"Human keratinocytes respond to interleukin-18:\u00a0implication for the course of chronic inflammatory skin diseases","volume":"124","author":"Wittmann","year":"2005","journal-title":"J Invest Dermatol"},{"key":"2023100310340270200_llac069-B24","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1046\/j.1523-1747.2000.02003.x","article-title":"Expression of IL-18 in human keratinocytes","volume":"114","author":"Companjen","year":"2000","journal-title":"J Invest Dermatol"},{"key":"2023100310340270200_llac069-B25","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1016\/j.jaci.2010.12.006","article-title":"Mite allergen is a danger signal for the skin via activation of inflammasome in keratinocytes","volume":"127","author":"Dai","year":"2011","journal-title":"J Allergy Clin Immunol"},{"key":"2023100310340270200_llac069-B26","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1111\/exd.13334","article-title":"Epidermal keratinocytes sense dsRNA via the NLRP3 inflammasome, mediating interleukin (IL)-1\u03b2 and IL-18 release","volume":"26","author":"Dai","year":"2017","journal-title":"Exp Dermatol"},{"key":"2023100310340270200_llac069-B27","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1038\/s41419-021-04159-9","article-title":"A novel function of NLRP3 independent of inflammasome as a key transcription factor of IL-33 in epithelial cells of atopic dermatitis","volume":"12","author":"Zheng","year":"2021","journal-title":"Cell Death Dis"},{"key":"2023100310340270200_llac069-B28","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1007\/978-1-62703-523-1_8","article-title":"ASC speck formation as a readout for inflammasome activation","volume":"1040","author":"Stutz","year":"2013","journal-title":"Methods Mol Biol Clifton NJ"},{"key":"2023100310340270200_llac069-B29","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1038\/nature25158","article-title":"Microglia-derived ASC specks cross-seed amyloid-\u03b2 in Alzheimer\u2019s disease","volume":"552","author":"Venegas","year":"2017","journal-title":"Nature"},{"key":"2023100310340270200_llac069-B30","doi-asserted-by":"crossref","first-page":"2157","DOI":"10.1016\/j.jid.2018.03.1523","article-title":"The major orphan forms of ichthyosis are characterized by systemic T-cell activation and Th-17\/Tc-17\/Th-22\/Tc-22 polarization in blood","volume":"138","author":"Czarnowicki","year":"2018","journal-title":"J Invest Dermatol"},{"key":"2023100310340270200_llac069-B31","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1016\/j.jaci.2009.06.009","article-title":"Com\u00e8l-Netherton syndrome defined as primary immunodeficiency","volume":"124","author":"Renner","year":"2009","journal-title":"J Allergy Clin Immunol"},{"key":"2023100310340270200_llac069-B32","first-page":"744","article-title":"Caspase-1 activity of stratum corneum and serum interleukin-18 level are increased in patients with Netherton syndrome","volume":"159","author":"Hosomi","year":"2008","journal-title":"Br J Dermatol"},{"key":"2023100310340270200_llac069-B33","doi-asserted-by":"crossref","first-page":"E649","DOI":"10.3390\/ijms20030649","article-title":"Interleukin-18 in health and disease","volume":"20","author":"Yasuda","year":"2019","journal-title":"Int J Mol Sci"},{"key":"2023100310340270200_llac069-B34","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1038\/378088a0","article-title":"Cloning of a new cytokine that induces IFN-gamma production by T cells","volume":"378","author":"Okamura","year":"1995","journal-title":"Nature"},{"key":"2023100310340270200_llac069-B35","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2021.808012","article-title":"The immunological impact of IL-1 family cytokines on the epidermal barrier","volume":"12","author":"Macleod","year":"2021","journal-title":"Front Immunol"},{"key":"2023100310340270200_llac069-B36","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1111\/bjd.20975","article-title":"Therapeutic potential of targeting interleukin-1 family cytokines in chronic inflammatory skin diseases","volume":"186","author":"Calabrese","year":"2022","journal-title":"Br J Dermatol"},{"key":"2023100310340270200_llac069-B37","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1002\/1529-0131(200103)44:3<550::AID-ANR103>3.0.CO;2-5","article-title":"Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still\u2019s disease","volume":"44","author":"Kawashima","year":"2001","journal-title":"Arthritis Rheum"},{"key":"2023100310340270200_llac069-B38","doi-asserted-by":"crossref","first-page":"1716","DOI":"10.1002\/1529-0131(200107)44:7<1716::AID-ART298>3.0.CO;2-I","article-title":"Interleukin-18 as a novel diagnostic marker and indicator of disease severity in adult-onset Still\u2019s disease","volume":"44","author":"Kawaguchi","year":"2001","journal-title":"Arthritis Rheum"},{"key":"2023100310340270200_llac069-B39","first-page":"2189","article-title":"Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still\u2019s disease","volume":"31","author":"Chen","year":"2004","journal-title":"J Rheumatol"},{"key":"2023100310340270200_llac069-B40","doi-asserted-by":"crossref","first-page":"2237","DOI":"10.1093\/rheumatology\/kew300","article-title":"Elevated serum levels of free interleukin-18 in adult-onset Still\u2019s disease","volume":"55","author":"Girard","year":"2016","journal-title":"Rheumatol Oxf Engl"},{"key":"2023100310340270200_llac069-B41","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1186\/s41927-019-0053-z","article-title":"Comparative study of interleukin-18 (IL-18) serum levels in adult onset Still\u2019s disease (AOSD) and systemic onset juvenile idiopathic arthritis (sJIA) and its use as a biomarker for diagnosis and evaluation of disease activity","volume":"3","author":"Kudela","year":"2019","journal-title":"BMC Rheumatol"},{"key":"2023100310340270200_llac069-B42","doi-asserted-by":"crossref","first-page":"910","DOI":"10.3390\/jcm10050910","article-title":"Different features of interleukin-37 and interleukin-18 as disase activity markers of adult-onset Still\u2019s disease","volume":"10","author":"Nam","year":"2021","journal-title":"J Clin Med"},{"key":"2023100310340270200_llac069-B43","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1272\/jnms.JNMS.2022_89-114","article-title":"Serum interleukin-18 provides a clue to the diagnosis of adult-onset Still\u2019s disease: findings from 6 Japanese patients with adult-onset Still\u2019s disease","volume":"89","author":"Ogita","year":"2022","journal-title":"J Nippon Med Sch Nippon Ika Daigaku Zasshi"},{"key":"2023100310340270200_llac069-B44","doi-asserted-by":"crossref","first-page":"3205","DOI":"10.1002\/art.23868","article-title":"Evidence for a pathogenetic role of interleukin-18 in cutaneous lupus erythematosus","volume":"58","author":"Wang","year":"2008","journal-title":"Arthritis Rheum"},{"key":"2023100310340270200_llac069-B45","article-title":"IL18-containing 5-gene signature distinguishes histologically identical dermatomyositis and lupus erythematosus skin lesions","volume":"5","author":"Tsoi","year":"2021","journal-title":"JCI Insight"},{"key":"2023100310340270200_llac069-B46","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1046\/j.1468-3083.2003.00647.x","article-title":"Serum levels of interleukin-18 and s-ICAM-1 in patients affected by psoriasis: preliminary considerations","volume":"17","author":"Gangemi","year":"2003","journal-title":"J Eur Acad Dermatol Venereol"},{"key":"2023100310340270200_llac069-B47","doi-asserted-by":"crossref","first-page":"e115","DOI":"10.1111\/jdv.13856","article-title":"High level of serum human interleukin-18 in a patient with pyogenic arthritis, pyoderma gangrenosum and acne syndrome","volume":"31","author":"Kanameishi","year":"2017","journal-title":"J Eur Acad Dermatol Venereol"},{"key":"2023100310340270200_llac069-B48","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.cyto.2017.10.007","article-title":"Longitudinal analysis of serum interleukin-18 in patients with familial Mediterranean fever carrying MEFV mutations in exon 10","volume":"104","author":"Wada","year":"2018","journal-title":"Cytokine"},{"key":"2023100310340270200_llac069-B49","doi-asserted-by":"crossref","first-page":"269\u00a0","DOI":"10.1186\/s13023-022-02430-6","article-title":"New developments in the molecular treatment of ichthyosis: review of the literature","volume":"17","author":"Joosten","year":"2022","journal-title":"Orphanet J Rare Dis"},{"key":"2023100310340270200_llac069-B50","doi-asserted-by":"crossref","first-page":"1947","DOI":"10.1038\/jid.2011.124","article-title":"Infliximab infusions for Netherton syndrome: sustained clinical improvement correlates with a reduction of thymic stromal lymphopoietin levels in the skin","volume":"131","author":"Fontao","year":"2011","journal-title":"J Invest Dermatol"},{"key":"2023100310340270200_llac069-B51","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1001\/jamadermatol.2019.4608","article-title":"Treatment of Netherton syndrome with dupilumab","volume":"156","author":"Steuer","year":"2020","journal-title":"JAMA Dermatol"},{"key":"2023100310340270200_llac069-B52","doi-asserted-by":"crossref","first-page":"e152","DOI":"10.1111\/jdv.16883","article-title":"Response to dupilumab in two children with Netherton syndrome: Improvement of pruritus and scaling","volume":"35","author":"S\u00fc\u00dfmuth","year":"2021","journal-title":"J Eur Acad Dermatol Venereol"},{"key":"2023100310340270200_llac069-B53","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/j.jdermsci.2021.03.003","article-title":"Successful dupilumab treatment for ichthyotic and atopic features of Netherton syndrome","volume":"102","author":"Murase","year":"2021","journal-title":"J Dermatol Sci"},{"key":"2023100310340270200_llac069-B54","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1111\/bjd.18892","article-title":"Successful treatment of Netherton syndrome with ustekinumab in a 15-year-old girl","volume":"183","author":"Volc","year":"2020","journal-title":"Br J Dermatol"},{"key":"2023100310340270200_llac069-B55","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1001\/jamadermatol.2020.1019","article-title":"Secukinumab therapy for Netherton syndrome","volume":"156","author":"Luchsinger","year":"2020","journal-title":"JAMA Dermatol"},{"key":"2023100310340270200_llac069-B56","doi-asserted-by":"crossref","first-page":"1476","DOI":"10.1016\/j.jaad.2020.07.054","article-title":"Duality of Netherton syndrome manifestations and response to ixekizumab","volume":"84","author":"Barbieux","year":"2021","journal-title":"J Am Acad Dermatol"},{"key":"2023100310340270200_llac069-B57","first-page":"840","article-title":"Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alfa (IL-18BP) in adult-onset Still\u2019s disease","volume":"77","author":"Gabay","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"2023100310340270200_llac069-B58","doi-asserted-by":"crossref","first-page":"1784","DOI":"10.1172\/JCI38177","article-title":"Epidermolysis bullosa simplex: a paradigm for disorders of tissue fragility","volume":"119","author":"Coulombe","year":"2009","journal-title":"J Clin Invest"},{"key":"2023100310340270200_llac069-B59","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1093\/carcin\/bgw063","article-title":"Keratin 8-deletion induced colitis predisposes to murine colorectal cancer enforced by the inflammasome and IL-22 pathway","volume":"37","author":"Misiorek","year":"2016","journal-title":"Carcinogenesis"},{"key":"2023100310340270200_llac069-B60","doi-asserted-by":"crossref","DOI":"10.1038\/ncomms7574","article-title":"Vimentin regulates activation of the NLRP3 inflammasome","volume":"6","author":"dos Santos","year":"2015","journal-title":"Nat Commun"},{"key":"2023100310340270200_llac069-B61","doi-asserted-by":"crossref","first-page":"e87","DOI":"10.1111\/jdv.15990","article-title":"Amino acid charge and epidermolysis bullosa simplex severity: genotype-phenotype correlations","volume":"34","author":"Ansai","year":"2020","journal-title":"J Eur Acad Dermatol Venereol"},{"key":"2023100310340270200_llac069-B62","doi-asserted-by":"crossref","DOI":"10.1111\/1346-8138.15476","article-title":"Mild epidermolytic ichthyosis with palmoplantar keratoderma due to the KRT1 mutation p.lle479Thr","volume":"47","author":"Murase","year":"2020","journal-title":"J Dermatol"}],"container-title":["Clinical and Experimental Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ced\/advance-article-pdf\/doi\/10.1093\/ced\/llac069\/48769703\/llac069.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ced\/article-pdf\/48\/3\/199\/51832505\/llac069.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ced\/article-pdf\/48\/3\/199\/51832505\/llac069.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T10:35:58Z","timestamp":1696329358000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ced\/article\/48\/3\/199\/6823796"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,11]]},"references-count":62,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2022,11,11]]},"published-print":{"date-parts":[[2023,3,1]]}},"URL":"https:\/\/doi.org\/10.1093\/ced\/llac069","relation":{},"ISSN":["0307-6938","1365-2230"],"issn-type":[{"type":"print","value":"0307-6938"},{"type":"electronic","value":"1365-2230"}],"subject":[],"published-other":{"date-parts":[[2023,3]]},"published":{"date-parts":[[2022,11,11]]}}}