{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,6]],"date-time":"2026-07-06T23:45:20Z","timestamp":1783381520307,"version":"3.54.6"},"reference-count":40,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2019,7,8]],"date-time":"2019-07-08T00:00:00Z","timestamp":1562544000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2019,7,8]],"date-time":"2019-07-08T00:00:00Z","timestamp":1562544000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Rheumatol"],"published-print":{"date-parts":[[2019,12]]},"DOI":"10.1186\/s12969-019-0338-1","type":"journal-article","created":{"date-parts":[[2019,7,8]],"date-time":"2019-07-08T13:03:25Z","timestamp":1562591005000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":44,"title":["Scoping review of biological treatment of deficiency of interleukin-36 receptor antagonist (DITRA) in children and adolescents"],"prefix":"10.1186","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5656-9165","authenticated-orcid":false,"given":"Toni","family":"Hospach","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabian","family":"Glowatzki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Friederike","family":"Blankenburg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dennis","family":"Conzelmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Stirnkorb","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris Sandra","family":"M\u00fcllersch\u00f6n","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"von den Driesch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa Maria","family":"K\u00f6hler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meino","family":"Rohlfs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Klein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabian","family":"Hauck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,7,8]]},"reference":[{"key":"338_CR1","doi-asserted-by":"crossref","unstructured":"Bal E, Lim AC, et al. Mutation in IL36RN impairs the processing and regulatory function of the interleukin-36-receptor antagonist and is associated with DITRA syndrome. Exp Dermatol. 2017.","DOI":"10.1111\/exd.13387"},{"key":"338_CR2","doi-asserted-by":"crossref","unstructured":"Boehner A, Navarini AA, et al. Generalized pustular psoriasis - a model disease for specific targeted immunotherapy, systematic review. Exp Dermatol. 2018.","DOI":"10.1111\/exd.13699"},{"issue":"8","key":"338_CR3","first-page":"1241","volume":"77","author":"N Bonekamp","year":"2018","unstructured":"Bonekamp N, Caorsi R, et al. High-dose ustekinumab for severe childhood deficiency of interleukin-36 receptor antagonist (DITRA). Ann Rheum Dis. 2018;77(8):1241\u20133.","journal-title":"Ann Rheum Dis"},{"issue":"12","key":"338_CR4","doi-asserted-by":"publisher","first-page":"2754","DOI":"10.1182\/blood-2011-08-374199","volume":"119","author":"YT Bryceson","year":"2012","unstructured":"Bryceson YT, Pende D, et al. A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes. Blood. 2012;119(12):2754\u201363.","journal-title":"Blood"},{"issue":"1","key":"338_CR5","doi-asserted-by":"publisher","first-page":"302","DOI":"10.1111\/bjd.13261","volume":"172","author":"R Carapito","year":"2015","unstructured":"Carapito R, Isidor B, et al. Homozygous IL36RN mutation and NSD1 duplication in a patient with severe pustular psoriasis and symptoms unrelated to deficiency of interleukin-36 receptor antagonist. Br J Dermatol. 2015;172(1):302\u20135.","journal-title":"Br J Dermatol"},{"issue":"12","key":"338_CR6","doi-asserted-by":"publisher","first-page":"e88","DOI":"10.1136\/annrheumdis-2017-212793","volume":"77","author":"B Cherqaoui Jr","year":"2018","unstructured":"Cherqaoui B Jr, Rossi-Semerano L, et al. Standard dose of Ustekinumab for childhood-onset deficiency of interleukin-36 receptor antagonist. Ann Rheum Dis. 2018;77(12):e88.","journal-title":"Ann Rheum Dis"},{"issue":"6","key":"338_CR7","doi-asserted-by":"publisher","first-page":"676","DOI":"10.1111\/ijd.12070","volume":"53","author":"SE Choon","year":"2014","unstructured":"Choon SE, Lai NM, et al. Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia. Int J Dermatol. 2014;53(6):676\u201384.","journal-title":"Int J Dermatol"},{"issue":"1","key":"338_CR8","doi-asserted-by":"publisher","first-page":"106","DOI":"10.1001\/jamadermatol.2016.3490","volume":"153","author":"KM Cordoro","year":"2017","unstructured":"Cordoro KM, Ucmak D, et al. Response to interleukin (IL)-17 inhibition in an adolescent with severe manifestations of IL-36 receptor antagonist deficiency (DITRA). JAMA Dermatol. 2017;153(1):106\u20138.","journal-title":"JAMA Dermatol"},{"issue":"3","key":"338_CR9","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1001\/archdermatol.2011.3014","volume":"148","author":"EW Cowen","year":"2012","unstructured":"Cowen EW, Goldbach-Mansky R. DIRA, DITRA, and new insights into pathways of skin inflammation: what's in a name? Arch Dermatol. 2012;148(3):381\u20134.","journal-title":"Arch Dermatol"},{"issue":"2","key":"338_CR10","doi-asserted-by":"publisher","first-page":"192","DOI":"10.1016\/j.jdcr.2017.08.019","volume":"4","author":"E Cuperus","year":"2018","unstructured":"Cuperus E, Koevoets R, et al. Juvenile interleukin-36 receptor antagonist deficiency (DITRA) with c.80T>C (p.Leu27Pro) mutation successfully treated with etanercept and acitretin. JAAD Case Rep. 2018;4(2):192\u20135.","journal-title":"JAAD Case Rep"},{"issue":"4","key":"338_CR11","doi-asserted-by":"publisher","first-page":"1025","DOI":"10.1038\/jid.2014.532","volume":"135","author":"AM D'Erme","year":"2015","unstructured":"D'Erme AM, Wilsmann-Theis D, et al. IL-36gamma (IL-1F9) is a biomarker for psoriasis skin lesions. J Invest Dermatol. 2015;135(4):1025\u201332.","journal-title":"J Invest Dermatol"},{"issue":"3","key":"338_CR12","doi-asserted-by":"publisher","first-page":"1440","DOI":"10.4049\/jimmunol.167.3.1440","volume":"167","author":"R Debets","year":"2001","unstructured":"Debets R, Timans JC, et al. Two novel IL-1 family members, IL-1 delta and IL-1 epsilon, function as an antagonist and agonist of NF-kappa B activation through the orphan IL-1 receptor-related protein 2. J Immunol. 2001;167(3):1440\u20136.","journal-title":"J Immunol"},{"issue":"2","key":"338_CR13","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1016\/j.berh.2014.05.005","volume":"28","author":"S Federici","year":"2014","unstructured":"Federici S, Gattorno M. A practical approach to the diagnosis of autoinflammatory diseases in childhood. Best Pract Res Clin Rheumatol. 2014;28(2):263\u201376.","journal-title":"Best Pract Res Clin Rheumatol"},{"issue":"7","key":"338_CR14","doi-asserted-by":"publisher","first-page":"1143","DOI":"10.1080\/19420862.2017.1353853","volume":"9","author":"R Ganesan","year":"2017","unstructured":"Ganesan R, Raymond EL, et al. Generation and functional characterization of anti-human and anti-mouse IL-36R antagonist monoclonal antibodies. MAbs. 2017;9(7):1143\u201354.","journal-title":"MAbs"},{"key":"338_CR15","doi-asserted-by":"crossref","unstructured":"Ho PH, Tsai TF. Successful treatment of refractory juvenile generalized pustular psoriasis with secukinumab monotherapy: a case report and review of published work. J Dermatol. 2018.","DOI":"10.1111\/1346-8138.14636"},{"issue":"4","key":"338_CR16","doi-asserted-by":"publisher","first-page":"1067","DOI":"10.1016\/j.jaci.2014.09.043","volume":"135","author":"S Hussain","year":"2015","unstructured":"Hussain S, Berki DM, et al. IL36RN mutations define a severe autoinflammatory phenotype of generalized pustular psoriasis. J Allergy Clin Immunol. 2015;135(4):1067\u20131070 e1069.","journal-title":"J Allergy Clin Immunol"},{"issue":"7","key":"338_CR17","doi-asserted-by":"publisher","first-page":"674","DOI":"10.1111\/1346-8138.12863","volume":"42","author":"H Jin","year":"2015","unstructured":"Jin H, Cho HH, et al. Clinical features and course of generalized pustular psoriasis in Korea. J Dermatol. 2015;42(7):674\u20138.","journal-title":"J Dermatol"},{"issue":"11","key":"338_CR18","doi-asserted-by":"publisher","first-page":"2634","DOI":"10.1038\/jid.2013.214","volume":"133","author":"A Korber","year":"2013","unstructured":"Korber A, Mossner R, et al. Mutations in IL36RN in patients with generalized pustular psoriasis. J Invest Dermatol. 2013;133(11):2634\u20137.","journal-title":"J Invest Dermatol"},{"issue":"5","key":"338_CR19","doi-asserted-by":"publisher","first-page":"936","DOI":"10.1093\/rheumatology\/kex510","volume":"57","author":"K Kostner","year":"2018","unstructured":"Kostner K, Prelog M, et al. Successful use of secukinumab in a 4-year-old patient with deficiency of interleukin-36 antagonist. Rheumatology (Oxford). 2018;57(5):936\u20138.","journal-title":"Rheumatology (Oxford)"},{"issue":"4","key":"338_CR20","doi-asserted-by":"publisher","first-page":"392","DOI":"10.1111\/ijd.13489","volume":"56","author":"BW Lau","year":"2017","unstructured":"Lau BW, Lim DZ, et al. Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia. Int J Dermatol. 2017;56(4):392\u20139.","journal-title":"Int J Dermatol"},{"issue":"2","key":"338_CR21","doi-asserted-by":"publisher","first-page":"132","DOI":"10.1016\/j.jdermsci.2014.08.007","volume":"76","author":"X Li","year":"2014","unstructured":"Li X, Chen M, et al. Mutation analysis of the IL36RN gene in Chinese patients with generalized pustular psoriasis with\/without psoriasis vulgaris. J Dermatol Sci. 2014;76(2):132\u20138.","journal-title":"J Dermatol Sci"},{"issue":"7","key":"338_CR22","doi-asserted-by":"publisher","first-page":"620","DOI":"10.1056\/NEJMoa1013068","volume":"365","author":"S Marrakchi","year":"2011","unstructured":"Marrakchi S, Guigue P, et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med. 2011;365(7):620\u20138.","journal-title":"N Engl J Med"},{"issue":"1","key":"338_CR23","first-page":"32","volume":"110","author":"AV Marzano","year":"2018","unstructured":"Marzano AV, Damiani G, et al. A dermatologic perspective on autoinflammatory diseases. Clin Exp Rheumatol. 2018;110(1):32\u20138.","journal-title":"Clin Exp Rheumatol"},{"issue":"5","key":"338_CR24","doi-asserted-by":"publisher","first-page":"473","DOI":"10.1001\/jamadermatol.2016.5705","volume":"153","author":"V Molho-Pessach","year":"2017","unstructured":"Molho-Pessach V, Alyan R, et al. Secukinumab for the treatment of deficiency of interleukin 36 receptor antagonist in an adolescent. JAMA Dermatol. 2017;153(5):473\u20135.","journal-title":"JAMA Dermatol"},{"issue":"5","key":"338_CR25","doi-asserted-by":"publisher","first-page":"534","DOI":"10.1159\/000449526","volume":"232","author":"AS Murthy","year":"2016","unstructured":"Murthy AS, Leslie K. Autoinflammatory skin disease: a review of concepts and applications to general dermatology. Dermatology. 2016;232(5):534\u201340.","journal-title":"Dermatology"},{"issue":"3","key":"338_CR26","doi-asserted-by":"publisher","first-page":"432","DOI":"10.1016\/j.ajhg.2011.07.022","volume":"89","author":"A Onoufriadis","year":"2011","unstructured":"Onoufriadis A, Simpson MA, et al. Mutations in IL36RN\/IL1F5 are associated with the severe episodic inflammatory skin disease known as generalized pustular psoriasis. Am J Hum Genet. 2011;89(3):432\u20137.","journal-title":"Am J Hum Genet"},{"issue":"25","key":"338_CR27","doi-asserted-by":"publisher","first-page":"6016","DOI":"10.1182\/blood-2011-12-398958","volume":"119","author":"J Pagel","year":"2012","unstructured":"Pagel J, Beutel K, et al. Distinct mutations in STXBP2 are associated with variable clinical presentations in patients with familial hemophagocytic lymphohistiocytosis type 5 (FHL5). Blood. 2012;119(25):6016\u201324.","journal-title":"Blood"},{"issue":"3","key":"338_CR28","doi-asserted-by":"publisher","first-page":"164","DOI":"10.1111\/dth.12325","volume":"29","author":"J Pan","year":"2016","unstructured":"Pan J, Qiu L, et al. Juvenile generalized pustular psoriasis with IL36RN mutation treated with short-term infliximab. Dermatol Ther. 2016;29(3):164\u20137.","journal-title":"Dermatol Ther"},{"issue":"3","key":"338_CR29","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1016\/j.det.2013.04.009","volume":"31","author":"JJ Rivas Bejarano","year":"2013","unstructured":"Rivas Bejarano JJ, Valdecantos WC. Psoriasis as autoinflammatory disease. Dermatol Clin. 2013;31(3):445\u201360.","journal-title":"Dermatol Clin"},{"issue":"2","key":"338_CR30","doi-asserted-by":"publisher","first-page":"279","DOI":"10.1016\/j.jaad.2011.01.032","volume":"67","author":"A Robinson","year":"2012","unstructured":"Robinson A, Van Voorhees AS, et al. Treatment of pustular psoriasis: from the medical Board of the National Psoriasis Foundation. J Am Acad Dermatol. 2012;67(2):279\u201388.","journal-title":"J Am Acad Dermatol"},{"issue":"4","key":"338_CR31","doi-asserted-by":"publisher","first-page":"e1043","DOI":"10.1542\/peds.2012-3935","volume":"132","author":"L Rossi-Semerano","year":"2013","unstructured":"Rossi-Semerano L, Piram M, et al. First clinical description of an infant with interleukin-36-receptor antagonist deficiency successfully treated with anakinra. Pediatrics. 2013;132(4):e1043\u20137.","journal-title":"Pediatrics"},{"issue":"3","key":"338_CR32","doi-asserted-by":"publisher","first-page":"424","DOI":"10.5021\/ad.2014.26.3.424","volume":"26","author":"HS Song","year":"2014","unstructured":"Song HS, Yun SJ, et al. Gene mutation analysis in a korean patient with early-onset and recalcitrant generalized pustular psoriasis. Ann Dermatol. 2014;26(3):424\u20135.","journal-title":"Ann Dermatol"},{"issue":"11","key":"338_CR33","doi-asserted-by":"publisher","first-page":"2514","DOI":"10.1038\/jid.2013.230","volume":"133","author":"K Sugiura","year":"2013","unstructured":"Sugiura K, Takemoto A, et al. The majority of generalized pustular psoriasis without psoriasis vulgaris is caused by deficiency of interleukin-36 receptor antagonist. J Invest Dermatol. 2013;133(11):2514\u201321.","journal-title":"J Invest Dermatol"},{"issue":"9","key":"338_CR34","doi-asserted-by":"publisher","first-page":"1811","DOI":"10.1016\/j.jid.2016.04.038","volume":"136","author":"M Tauber","year":"2016","unstructured":"Tauber M, Bal E, et al. IL36RN mutations affect protein expression and function: a basis for genotype-phenotype correlation in pustular diseases. J Invest Dermatol. 2016;136(9):1811\u20139.","journal-title":"J Invest Dermatol"},{"issue":"3","key":"338_CR35","doi-asserted-by":"publisher","first-page":"646","DOI":"10.1111\/bjd.13012","volume":"171","author":"M Tauber","year":"2014","unstructured":"Tauber M, Viguier M, et al. Partial clinical response to anakinra in severe palmoplantar pustular psoriasis. Br J Dermatol. 2014;171(3):646\u20139.","journal-title":"Br J Dermatol"},{"issue":"14","key":"338_CR36","doi-asserted-by":"publisher","first-page":"13677","DOI":"10.1074\/jbc.M400117200","volume":"279","author":"JE Towne","year":"2004","unstructured":"Towne JE, Garka KE, et al. Interleukin (IL)-1F6, IL-1F8, and IL-1F9 signal through IL-1Rrp2 and IL-1RAcP to activate the pathway leading to NF-kappaB and MAPKs. J Biol Chem. 2004;279(14):13677\u201388.","journal-title":"J Biol Chem"},{"issue":"1","key":"338_CR37","doi-asserted-by":"publisher","first-page":"66","DOI":"10.7326\/0003-4819-153-1-201007060-00030","volume":"153","author":"M Viguier","year":"2010","unstructured":"Viguier M, Guigue P, et al. Successful treatment of generalized pustular psoriasis with the interleukin-1-receptor antagonist Anakinra: lack of correlation with IL1RN mutations. Ann Intern Med. 2010;153(1):66\u20137.","journal-title":"Ann Intern Med"},{"issue":"1","key":"338_CR38","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1007\/s00403-015-1611-x","volume":"308","author":"TS Wang","year":"2016","unstructured":"Wang TS, Chiu HY, et al. Correlation of IL36RN mutation with different clinical features of pustular psoriasis in Chinese patients. Arch Dermatol Res. 2016;308(1):55\u201363.","journal-title":"Arch Dermatol Res"},{"issue":"3","key":"338_CR39","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1016\/j.jdermsci.2016.11.008","volume":"85","author":"Y Wang","year":"2017","unstructured":"Wang Y, Cheng R, et al. Clinical profiles of pediatric patients with GPP alone and with different IL36RN genotypes. J Dermatol Sci. 2017;85(3):235\u201340.","journal-title":"J Dermatol Sci"},{"issue":"2","key":"338_CR40","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1684\/ejd.2018.3245","volume":"28","author":"T Zhu","year":"2018","unstructured":"Zhu T, Jin H, et al. Association of IL36RN mutations with clinical features, therapeutic response to acitretin, and frequency of recurrence in patients with generalized pustular psoriasis. Eur J Dermatol. 2018;28(2):217\u201324.","journal-title":"Eur J Dermatol"}],"container-title":["Pediatric Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-019-0338-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s12969-019-0338-1\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-019-0338-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,7,6]],"date-time":"2020-07-06T23:16:51Z","timestamp":1594077411000},"score":1,"resource":{"primary":{"URL":"https:\/\/ped-rheum.biomedcentral.com\/articles\/10.1186\/s12969-019-0338-1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,7,8]]},"references-count":40,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2019,12]]}},"alternative-id":["338"],"URL":"https:\/\/doi.org\/10.1186\/s12969-019-0338-1","relation":{},"ISSN":["1546-0096"],"issn-type":[{"value":"1546-0096","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,7,8]]},"assertion":[{"value":"21 February 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"12 June 2019","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 July 2019","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Name of the ethics committee: Landes\u00e4rztekammer Baden-W\u00fcrttemberg, Liebknechtstr. 33, 70565 Stuttgart.Approval and consent was obtained on February 5th, 2019.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Informed consent was obtained of both parents in written and of the pediatric patient in oral form for publishing the case report and the pictures.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"37"}}