{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T12:23:43Z","timestamp":1791894223352,"version":"4.3.4"},"reference-count":19,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2022,2,26]],"date-time":"2022-02-26T00:00:00Z","timestamp":1645833600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2022,2,26]],"date-time":"2022-02-26T00:00:00Z","timestamp":1645833600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"funder":[{"DOI":"10.13039\/100008272","name":"Novartis Pharmaceuticals Corporation","doi-asserted-by":"publisher","award":["SP0036673"],"award-info":[{"award-number":["SP0036673"]}],"id":[{"id":"10.13039\/100008272","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arch Dermatol Res"],"DOI":"10.1007\/s00403-022-02325-3","type":"journal-article","created":{"date-parts":[[2022,2,26]],"date-time":"2022-02-26T08:02:36Z","timestamp":1645862556000},"page":"305-315","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Secukinumab responses vary across the spectrum of congenital ichthyosis in adults"],"prefix":"10.1007","volume":"315","clinical-trial-number":[{"clinical-trial-number":"nct03041038","registry":"10.18810\/clinical-trials-gov"}],"author":[{"given":"Rachel","family":"Lefferdink","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1206-9561","authenticated-orcid":false,"given":"Stephanie M.","family":"Rangel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margot","family":"Chima","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erin","family":"Ibler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana B.","family":"Pavel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"HeeJin","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedict","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hajar","family":"Abu-Zayed","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianni","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn","family":"Jackson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giselle","family":"Singer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keith A.","family":"Choate","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Guttman-Yassky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy S.","family":"Paller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,2,26]]},"reference":[{"key":"2325_CR1","doi-asserted-by":"publisher","unstructured":"AS, Renert-Yuval Y, Suprun M, Esaki H, Oliva M, Huynh TN, et al. (2017) An IL-17\u2013dominant immune profile is shared across the major orphan forms of ichthyosis. J Allergy Clin Immunol 139:152-165. https:\/\/doi.org\/10.1016\/j.jaci.2016.07.019","DOI":"10.1016\/j.jaci.2016.07.019"},{"key":"2325_CR2","doi-asserted-by":"publisher","first-page":"604","DOI":"10.1016\/j.jaci.2018.03.021","volume":"143","author":"K Malik","year":"2019","unstructured":"Malik K, He H, Huynh TN, Tran G, Mueller K, Doytcheva K et al (2019) Ichthyosis molecular fingerprinting shows profound TH17 skewing and a unique barrier genomic signature. J Allergy Clin Immunol 143:604\u2013618. https:\/\/doi.org\/10.1016\/j.jaci.2018.03.021","journal-title":"J Allergy Clin Immunol"},{"key":"2325_CR3","doi-asserted-by":"publisher","first-page":"2157","DOI":"10.1016\/j.jid.2018.03.1523","volume":"138","author":"T Czarnowicki","year":"2018","unstructured":"Czarnowicki T, He H, Leonard A, Malik K, Magidi S, Rangel S et al (2018) 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. J Invest Dermatol 138:2157\u20132167. https:\/\/doi.org\/10.1016\/j.jid.2018.03.1523","journal-title":"J Invest Dermatol"},{"key":"2325_CR4","doi-asserted-by":"publisher","first-page":"400","DOI":"10.1016\/j.jaad.2015.05.013","volume":"73","author":"D Tha\u00e7i","year":"2015","unstructured":"Tha\u00e7i D, Blauvelt A, Reich K, Tsai TF, Vanaclocha F, Kingo K et al (2015) Secukinumab is superior to ustekinumab in clearing skin of subjects with moderate to severe plaque psoriasis: CLEAR, a randomized controlled trial. J Am Acad Dermatol 73:400\u2013409. https:\/\/doi.org\/10.1016\/j.jaad.2015.05.013","journal-title":"J Am Acad Dermatol"},{"key":"2325_CR5","doi-asserted-by":"publisher","first-page":"1834","DOI":"10.1016\/j.jid.2017.04.037","volume":"137","author":"NV Marukian","year":"2017","unstructured":"Marukian NV, Deng Y, Gan G, Ren I, Thermidor W, Craiglow BG et al (2017) Establishing and validating an ichthyosis severity index. J Invest Dermatol 137:1834\u20131841. https:\/\/doi.org\/10.1016\/j.jid.2017.04.037","journal-title":"J Invest Dermatol"},{"key":"2325_CR6","doi-asserted-by":"publisher","first-page":"1087","DOI":"10.1111\/j.1365-2133.2011.10510","volume":"165","author":"C Bodemer","year":"2011","unstructured":"Bodemer C, Bourrat E, Mazereeuw-Hautier J, Boralevi F, Barbarot S, Bessis D, Blanchet-Bardon C et al (2011) Short- and medium-term efficacy of specific hydrotherapy in inherited ichthyosis. Br J Dermatol 165:1087\u20131094. https:\/\/doi.org\/10.1111\/j.1365-2133.2011.10510","journal-title":"Br J Dermatol"},{"key":"2325_CR7","doi-asserted-by":"publisher","first-page":"771","DOI":"10.1111\/pde.14221","volume":"37","author":"TR Erickson","year":"2020","unstructured":"Erickson TR, Murphrey MB, Abu-Zayed H, Wu B, Ibler E, Rangel SM et al (2020) Transepidermal water loss in the orphan forms of ichthyosis. Pediatr Dermatol 37:771\u2013773. https:\/\/doi.org\/10.1111\/pde.14221","journal-title":"Pediatr Dermatol"},{"key":"2325_CR8","doi-asserted-by":"publisher","first-page":"587","DOI":"10.1111\/j.1365-2133.2009.09586","volume":"162","author":"S Elman","year":"2009","unstructured":"Elman S, Hynan LS, Gabriel V, Mayo MJ (2009) The 5-D itch scale: a new measure of pruritus. Br J Dermatol 162:587\u2013593. https:\/\/doi.org\/10.1111\/j.1365-2133.2009.09586","journal-title":"Br J Dermatol"},{"key":"2325_CR9","doi-asserted-by":"publisher","first-page":"997","DOI":"10.1111\/j.1365-2133.2008.08832","volume":"159","author":"MK Basra","year":"2008","unstructured":"Basra MK, Fenech R, Gatt RM, Salek MS, Finlay AY (2008) The Dermatology Life Quality Index 1994\u20132007: a comprehensive review of validation data and clinical results. Br J Dermatol 159:997\u20131035. https:\/\/doi.org\/10.1111\/j.1365-2133.2008.08832","journal-title":"Br J Dermatol"},{"key":"2325_CR10","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1016\/j.jaad.2013.01.022","volume":"69","author":"I Dreyfus","year":"2013","unstructured":"Dreyfus I, Ta\u00efeb C, Barbarot S, Maza A, Galera I, Bourrat E, et al. et al (2013) iQoL-32: a new ichthyosis-specific measure of quality of life. J Am Acad Dermatol 69:82\u201387. https:\/\/doi.org\/10.1016\/j.jaad.2013.01.022","journal-title":"J Am Acad Dermatol"},{"key":"2325_CR11","doi-asserted-by":"publisher","first-page":"750","DOI":"10.1016\/j.jaci.2019.04.029","volume":"144","author":"JG Krueger","year":"2019","unstructured":"Krueger JG, Wharton KA Jr, Schlitt T, Suprun M, Torene RI, Jiang X et al (2019) IL-17A inhibition by secukinumab induces early clinical, histopathologic, and molecular resolution of psoriasis. J Allergy Clin Immunol 144:750\u2013763. https:\/\/doi.org\/10.1016\/j.jaci.2019.04.029","journal-title":"J Allergy Clin Immunol"},{"key":"2325_CR12","doi-asserted-by":"publisher","unstructured":"Hern\u00e1ndez-Mart\u00edn A, Kennedy-Batalla R, Ca\u00f1edo E, Gonz\u00e1lez-Sarmiento R, Kennedy-Batalla Rebeca, Mart\u00ednez-Bonet Marta, et al. (2019) Imbalance in T-helper 17 cells and targeted therapy in an infant with SAM-like syndrome.\u00a0N Engl J Med 381:2176\u20132178. https:\/\/doi.org\/10.1056\/NEJMc1908531","DOI":"10.1056\/NEJMc1908531"},{"key":"2325_CR13","doi-asserted-by":"publisher","unstructured":"Paller AS. (2020) Pathogenesis-based therapy with repurposed biologics for monogenic inflammatory skin disorders. JAMA Dermatol 156:839\u201341. https:\/\/doi.org\/10.1001\/jamadermatol.2020.1018","DOI":"10.1001\/jamadermatol.2020.1018"},{"key":"2325_CR14","doi-asserted-by":"publisher","unstructured":"Luchsinger I, Kn\u00f6pfel N, Theiler M, Bonnet des Claustres M, Barbieux C, Schwieger-Briel A, et al. (2020) Secukinumab therapy for Netherton syndrome. JAMA Dermatol156:907\u201311. https:\/\/doi.org\/10.1001\/jamadermatol.2020.1019","DOI":"10.1001\/jamadermatol.2020.1019"},{"key":"2325_CR15","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1016\/j.jdcr.2020.04.025","volume":"6","author":"SK Blanchard","year":"2020","unstructured":"Blanchard SK, Prose NS (2020) Successful use of secukinumab in Netherton syndrome. JAAD Case Rep 6:577\u2013578. https:\/\/doi.org\/10.1016\/j.jdcr.2020.04.025","journal-title":"JAAD Case Rep"},{"key":"2325_CR16","doi-asserted-by":"publisher","unstructured":"Paller AS, Czarnowicki T, Renert-Yuval Y, Holland K, Huynh T, Sadlier M, et al. (2018) The spectrum of manifestations in desmoplakin gene (DSP) spectrin repeat 6 domain mutations: Immunophenotyping and response to ustekinumab. J Am Acad Dermatol 78:498\u2013505.e2. https:\/\/doi.org\/10.1016\/j.jaad.2017.10.026","DOI":"10.1016\/j.jaad.2017.10.026"},{"key":"2325_CR17","doi-asserted-by":"publisher","unstructured":"Sun Q, Wine Lee L, Hall EK, Choate KA, Elder RW. (2021) Hair and skin predict cardiomyopathies: Carvajal and erythrokeratodermia cardiomyopathy syndromes. Pediatr Dermatol 38:31\u201338. https:\/\/doi.org\/10.1111\/pde.14478","DOI":"10.1111\/pde.14478"},{"key":"2325_CR18","doi-asserted-by":"publisher","unstructured":"Frommherz LH, Schempp CM, Has C. (2021) Secukinumab for the treatment of SAM syndrome associated with desmoglein-1 deficiency. Br J Dermatol 184:770\u2013772. https:\/\/doi.org\/10.1111\/bjd.19684","DOI":"10.1111\/bjd.19684"},{"key":"2325_CR19","doi-asserted-by":"publisher","unstructured":"Steinhoff, M,\u00a0Al-Marri, F,\u00a0Al Chalabi, R,\u00a0Gieler, U,\u00a0Buddenkotte, J.\u00a0(2021) Recalcitrant erythrodermic ichthyosis with atopic dermatitis successfully treated with dupilumab in combination with guselkumab. Skin Health Dis.e87. https:\/\/doi.org\/10.1002\/ski2.87","DOI":"10.1002\/ski2.87"}],"container-title":["Archives of Dermatological Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00403-022-02325-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00403-022-02325-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00403-022-02325-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,2,19]],"date-time":"2023-02-19T17:27:31Z","timestamp":1676827651000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00403-022-02325-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,2,26]]},"references-count":19,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2023,3]]}},"alternative-id":["2325"],"URL":"https:\/\/doi.org\/10.1007\/s00403-022-02325-3","relation":{},"ISSN":["1432-069X"],"issn-type":[{"value":"1432-069X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,2,26]]},"assertion":[{"value":"8 September 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 January 2022","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 January 2022","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"26 February 2022","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Drs. Lefferdink, Chima, Ibler, Pavel, Kim, Wu, and Rangel, and Ms. Abu-Zayed, Wu, Jackson, and Singer declare no conflicts. Dr. Choate has been an investigator for Alderya, Mayne, Galderma, and Regeneron and consultant with honorarium for AbbVie, Eli Lilly, Janssen, KrystalBio, Lifemax, Mayne, and Timber. Dr. Guttman-Yassky has been a researcher\/consultant for AbbVie, Anacor, AnaptysBio, Asana Biosciences, Botanix, Celgene, DBV, Dermira, DS Biopharma, Escalier, Galderma, Glenmark, Innovaderm, Janssen, Kyowa Kirin, Leo Pharma, Lilly, MedImmune\/AstraZeneca, Mitsubishi Tanabe, Novan, Novartis, Pfizer, Promius, Ralexar, Regeneron, Sanofi-Aventis, Stiefel\/GlaxoSmithKline (GSK), UCB, and Vitae. Dr. Paller has been an investigator for AbbVie, AnaptysBio, Eli Lilly, Incyte, Janssen, KrystalBio, Novartis, Regeneron, and UCB and a consultant with honorarium for Abbvie, Abeona, Alcimed, Almirall, Amagma, Anaptysbio, Arena, Azitra, BiomX, Boehringer Ingelheim, Castle Biosciences, Catawba, Dermira, Eli Lilly, Exicure, Forte, Kamari, Leo, Lifemax, NAOS, Novartis, Pfizer, Phoenix, Pierre Fabre, Regeneron, Sanofi\/Genzyme, Seanergy, Trifecta, and UCB. She has served on Data Safety Monitoring Boards for AbbVie, Bausch, Galderma, and Novan.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"This study was reviewed and approved by the institutional review boards of Northwestern University and Icahn School of Medicine at Mount Sinai Medical Center. IRB #: STU00202656\/STU00202022 (NU); 17\u201300126 (MS).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval"}},{"value":"Informed consent was obtained from all individual participants included in the study.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to participate"}}]}}