[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-459096-105":59,"doc-detail-459096-en":130},{"code":4,"msg":5,"data":6},0,"success",[7,13,18,23,28,33,38,43,48,51,55],{"id":8,"doc_module":4,"doc_module_name":9,"category_name":10,"show_sort_weight":11,"slug":12},1,"Document","Story & Novel",90,"story-novel",{"id":14,"doc_module":4,"doc_module_name":9,"category_name":15,"show_sort_weight":16,"slug":17},2,"Literature",80,"literature",{"id":19,"doc_module":4,"doc_module_name":9,"category_name":20,"show_sort_weight":21,"slug":22},4,"Exam",70,"exam",{"id":24,"doc_module":4,"doc_module_name":9,"category_name":25,"show_sort_weight":26,"slug":27},5,"Comic",60,"comic",{"id":29,"doc_module":4,"doc_module_name":9,"category_name":30,"show_sort_weight":31,"slug":32},6,"Technology",50,"technology",{"id":34,"doc_module":4,"doc_module_name":9,"category_name":35,"show_sort_weight":36,"slug":37},7,"Healthcare",40,"healthcare",{"id":39,"doc_module":4,"doc_module_name":9,"category_name":40,"show_sort_weight":41,"slug":42},8,"Research & Report",30,"research-report",{"id":44,"doc_module":4,"doc_module_name":9,"category_name":45,"show_sort_weight":46,"slug":47},9,"Religion & Spirituality",20,"religion-spirituality",{"id":46,"doc_module":4,"doc_module_name":9,"category_name":49,"show_sort_weight":46,"slug":50},"World Cup","world-cup",{"id":52,"doc_module":4,"doc_module_name":9,"category_name":53,"show_sort_weight":52,"slug":54},10,"Lifestyle","lifestyle",{"id":56,"doc_module":4,"doc_module_name":9,"category_name":57,"show_sort_weight":24,"slug":58},19,"General","general",{"code":4,"msg":60,"data":61},"ok",{"site_id":62,"language":63,"slug":64,"title":65,"keywords":66,"description":67,"schema_data":68,"social_meta":123,"head_meta":125,"extra_data":127,"updated_unix":129},105,"en","severe-resistant-generalized-pustular-psoriasis-associated-inflammatory-syndrome-successfully-treated-with-adalimumab-a-case-report","Severe, Resistant Generalized Pustular Psoriasis-Associated Inflammatory Syndrome Successfully Treated With Adalimumab - A Case Report","","Generalized pustular psoriasis (GPP) is a rare, severe psoriasis variant, especially in pediatric patients, marked by recurrent fever, malaise, and sterile pustules. The report describes an eight-year-old girl with genetically confirmed GPP, carrying heterozygous IL36RN and CARD14 mutations. After prior therapies failed, adalimumab combined with methotrexate achieved sustained remission. The case supports genetic testing and suggests biologic agents as a management option for pediatric GPP, particularly in resistant disease.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/severe-resistant-generalized-pustular-psoriasis-associated-inflammatory-syndrome-successfully-treated-with-adalimumab-a-case-report/459096/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/severe-resistant-generalized-pustular-psoriasis-associated-inflammatory-syndrome-successfully-treated-with-adalimumab-a-case-report/459096.png","ImageObject",300,407,{"name":92,"@type":93},"CatatanPagi","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-11-01","2026-09-30",true,{"@type":102,"interactionType":103,"userInteractionCount":34},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What defines generalized pustular psoriasis (GPP) in this case?","Question",{"text":112,"@type":113},"GPP is described as a rare and severe psoriasis variant with recurrent episodes of fever, malaise, and sterile pustules, often with systemic inflammation. The article notes these features in the pediatric patient.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"Which genetic mutations were identified and why are they important?",{"text":117,"@type":113},"The patient had heterozygous mutations in IL36RN and CARD14 identified by whole-exome sequencing. The report presents these findings as supporting GPP’s genetic basis and diagnostic value.",{"name":119,"@type":110,"acceptedAnswer":120},"How was the patient treated and what was the outcome?",{"text":121,"@type":113},"Initial management included prednisolone and anakinra, followed by a flare during hospitalization. Sustained remission was achieved only after treatment with adalimumab combined with methotrexate.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},459096,1791080942,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":34,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":24,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144,"preview_page_count":4},962090894170,"https://ap-avatar.wpscdn.com/davatar_6f874abed73319feea01a86fa6f0fab8","Review began 05/27/2025  \nReview ended 11/26/2025  \nPublished 12/09/2025  \n© Copyright 2025  \nBourousis et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CCBY 4.0. , which permits unrestricted use , distribution , and reproduction in any medium , provided the original author and source are credited.  \nDOI: 10.7759/cureus.98830  \nOpen Access Case Report  \nSevere, Resistant Generalized Pustular PsoriasisAssociated Inflammatory Syndrome Successfully Treated With Adalimumab: A Case Report  \nEvangelos Bourousis 1, Marina Triantafyllia Kotzamani 2, Despoina N. Maritsi 2  \n1. Paediatrics and Child Health, Paediatric Hospital, Athens, GRC 2. Paediatrics, National and Kapodistrian University of Athens, Athens, GRC  \nCorresponding author: Marina Triantafyllia Kotzamani, [kotza.mari.tria.94@gmail.com](kotza.mari.tria.94@gmail.com)  \nAbstract  \nGeneralized pustular psoriasis (GPP) is a rare and severe variant of psoriasis, especially in pediatric patients, characterized by recurrent episodes of fever, malaise, and sterile pustules. We present the case of an eightyear-old girl with genetically confirmed GPP, carrying heterozygous mutations in IL36RN and CARD14, successfully treated with adalimumab in combination with methotrexate. Despite previous therapies, sustained remission was achieved only with this regimen. This case highlights the importance of genetic testing and the potential role of biologic agents in the management of pediatric GPP.  \nCategories: Genetics, Rheumatology, Pediatrics  \nKeywords: adalimumab (humira), generalized pustular psoriasis (gpp), genetics of psoriasis, pediatric psoriasis, pediatric rheumatology  \nIntroduction  \nPsoriasis is a chronic, immune-mediated inflammatory disease affecting approximately 2-3% of the global population [1] . Among its clinical variants, generalized pustular psoriasis (GPP) is one of the rarest and most severe, accounting for less than 1% of psoriasis cases [2] . GPP is characterized by recurrent flares of widespread sterile pustules on erythematous skin, often accompanied by systemic inflammation, including high fever, malaise, arthralgia, and laboratory evidence of systemic inflammation [3] .  \nChildhood-onset GPP is particularly uncommon, with most cases presenting within the first year of life [4] . The disease may follow a relapsing course with potentially life-threatening complications such as sepsis, hepatic dysfunction, and electrolyte imbalance if untreated [5] . Early diagnosis is therefore crucial but remains challenging due to clinical overlap with severe infections and other pustular dermatoses.  \nRecent advances have identified a strong genetic component in GPP. Mutations in the IL36RN gene, first described in 2011 [6], lead to defective interleukin-36 (IL-36) receptor antagonism and uncontrolled proinflammatory signaling. Additional mutations such as CARD14, BTN3A3, AP1S3, and SERPINA1 have also been implicated [6] . These discoveries highlight GPP as a distinct autoinflammatory disease entity, rather than merely a variant of plaque psoriasis.  \nTraditional therapies, including systemic corticosteroids, cyclosporine, methotrexate, and retinoids, have shown variable efficacy and are often associated with significant adverse effects in children [7] . The introduction of biologic agents, particularly tumor necrosis factor-alpha (TNF-α) inhibitors and, more recently, IL-36 receptor antagonists, has provided promising new options, although high-quality pediatric data remain limited [8,9] .  \nHere, we present the case of an eight-year-old girl with genetically confirmed GPP due to IL36RN and CARD14 mutations, who demonstrated dramatic and sustained clinical remission following treatment with adalimumab. This case emphasizes the diagnostic value of genetic testing and the potential role of biologics in pediatric GPP.  \nCase Presentation  \nAn eight-year-old Afghan girl, born from a non-consanguineous marriage, presented","cbCaioFDMP2LVf4r","https://ap.wps.com/l/cbCaioFDMP2LVf4r","pdf",4035848,"English","# Abstract\n# Introduction\n# Case Presentation","[{\"question\":\"What defines generalized pustular psoriasis (GPP) in this case?\",\"answer\":\"GPP is described as a rare and severe psoriasis variant with recurrent episodes of fever, malaise, and sterile pustules, often with systemic inflammation. The article notes these features in the pediatric patient.\"},{\"question\":\"Which genetic mutations were identified and why are they important?\",\"answer\":\"The patient had heterozygous mutations in IL36RN and CARD14 identified by whole-exome sequencing. The report presents these findings as supporting GPP’s genetic basis and diagnostic value.\"},{\"question\":\"How was the patient treated and what was the outcome?\",\"answer\":\"Initial management included prednisolone and anakinra, followed by a flare during hospitalization. Sustained remission was achieved only after treatment with adalimumab combined with methotrexate.\"}]","Severe, Resistant Generalized Pustular Psoriasis-Associated Inflammatory Syndrome Successfully Treated With Adalimumab - A Case Report | PDF",1790755196,13]