{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T16:58:43Z","timestamp":1785257923573,"version":"3.55.0"},"reference-count":18,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T00:00:00Z","timestamp":1780876800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T00:00:00Z","timestamp":1780876800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Case Rep Dermatol"],"abstract":"<jats:p>Introduction: Acute generalized exanthematous pustulosis (AGEP) is a rare but severe cutaneous adverse drug reaction characterized by the sudden onset of widespread sterile pustules on an erythematous and edematous base, often accompanied by fever and neutrophilia. Over 90% of cases are drug-induced. Its management can be particularly challenging in patients with autoimmune diseases such as systemic lupus erythematosus (SLE), where cutaneous manifestations and immune dysregulation may confound clinical assessment. Case Presentation: We report a case of severe hydroxychloroquine-induced AGEP in a patient with active SLE. The disease was refractory to systemic corticosteroids and further worsened after intravenous immunoglobulin infusion. Given the emerging role of interleukin (IL)-36 pathway dysregulation in pustular dermatoses, the patient was treated with spesolimab, a monoclonal antibody targeting the IL-36 receptor, resulting in rapid defervescence and near-complete resolution of pustules within days. Conclusion: This case underscores the need for prompt recognition of drug-induced AGEP in patients receiving antimalarials or other immunomodulatory agents for connective tissue diseases. Crosstalk between IL-36 signaling and neutrophil extracellular traps may amplify inflammation, linking AGEP with autoimmune pathology. IL-36 inhibition with spesolimab represents a potential rescue therapy for severe, treatment-refractory AGEP, particularly in patients with underlying autoimmune disorders where conventional therapies fail.<\/jats:p>","DOI":"10.1159\/000552623","type":"journal-article","created":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T16:03:49Z","timestamp":1780934629000},"page":"455-462","source":"Crossref","is-referenced-by-count":0,"title":["When Conventional Therapy Fails: Interleukin-36 Inhibition for Severe Hydroxychloroquine-Induced Acute Generalized Exanthematous Pustulosis in Systemic Lupus Erythematosus \u2013 A Case Report"],"prefix":"10.1159","volume":"18","author":[{"given":"Lu","family":"Yang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiayi","family":"Ying","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiuxiu","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1302-5667","authenticated-orcid":false,"given":"Chengfeng","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruiming","family":"Hu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ning","family":"Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2026,6,8]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1007\/s40257-023-00779-3"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.3390\/jcm11020397"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1007\/s40257-021-00654-z"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1111\/dth.13380"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.16903"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2013.44"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1016\/j.intimp.2018.03.011"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.3390\/cells10061469"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1172\/JCI162088"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.00746"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2025.01.046"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2025.04.007"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1093\/ced\/llae144"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1056\/nejmoa2111563"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.64474"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1080\/09546634.2025.2451811"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2025.1581996"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.17449"}],"container-title":["Case Reports in Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/article-pdf\/doi\/10.1159\/000552623","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T16:00:58Z","timestamp":1785254458000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000552623"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,8]]},"references-count":18,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,1,1]]}},"URL":"https:\/\/doi.org\/10.1159\/000552623","archive":["Portico"],"relation":{"is-supplemented-by":[{"id-type":"doi","id":"10.6084\/m9.figshare.32602635","asserted-by":"subject"}]},"ISSN":["1662-6567"],"issn-type":[{"value":"1662-6567","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,8]]}}}