{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T10:44:30Z","timestamp":1785926670538,"version":"3.56.0"},"reference-count":108,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T00:00:00Z","timestamp":1785888000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Clin. Med.","JCM"],"abstract":"<jats:p>Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening, chronic cutaneous inflammatory disease characterized by unpredictable, recurrent acute flares of painful sterile pustules on a widespread erythematous background. In addition to cutaneous manifestations, patients may experience fever, pruritus, pain, chills, and general malaise, which may be further complicated by secondary infection, sepsis, and organ failure, thus requiring urgent medical treatment and, in some cases, hospitalization. Prompt therapeutic management of the acute phase is crucial for severe cases, and proactive treatment to prevent flares should always be considered. However, early recognition of acute flares can be challenging due to the low frequency of the disease, the rapid onset of flares, the lack of hematological biomarkers and the absence of standardized diagnostic criteria. Moreover, despite the approval of new targeted therapies, there are still several unmet needs, as these treatments are highly expensive, not always readily available, and may have limited efficacy in patients with advanced or complicated disease. For these reasons, multidisciplinary round-table discussions and shared diagnostic and therapeutic algorithms involving dermatologists, who are responsible for diagnosing and treating GPP, and other medical specialists are desirable to facilitate prompt referral to dermatologists for accurate diagnosis and appropriate treatment. We report the updated literature discussed during a multidisciplinary meeting with the aim of providing practice recommendations for clinicians involved in GPP management.<\/jats:p>","DOI":"10.3390\/jcm15156087","type":"journal-article","created":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T09:16:22Z","timestamp":1785921382000},"page":"6087","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical Practice Recommendations for Non-Dermatologists on the Diagnostic Suspicion of GPP"],"prefix":"10.3390","volume":"15","author":[{"given":"Antonella","family":"Di Cesare","sequence":"first","affiliation":[{"name":"Dermatology Unit, USL Toscana Centro, Ospedale P. Palagi, Viale Michelangelo, 41, 50125 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4227-7847","authenticated-orcid":false,"given":"Elia","family":"Rosi","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Health Sciences, University of Florence, University of Florence Ospedale P. Palagi, Viale Michelangelo, 41, 50125 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annalisa","family":"Cavallo","sequence":"additional","affiliation":[{"name":"Infectious and Tropical Diseases Unit, AOU Azienda Ospedaliero Universitaria Careggi, 50134 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2722-6475","authenticated-orcid":false,"given":"Serena","family":"Guiducci","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, Section of Rheumatology, AOU Careggi, University of Florence, 50134 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-4748-8954","authenticated-orcid":false,"given":"Anna Lucia","family":"Marigliano","sequence":"additional","affiliation":[{"name":"Pharmaceutical Governance and Appropriateness of Prescription Unit, Drug Department, USL Toscana Centro, 59100 Prato, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1201-5715","authenticated-orcid":false,"given":"Simone","family":"Vanni","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, High Dependency Unit, Careggi University Hospital, University of Florence, 50134 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5997-2045","authenticated-orcid":false,"given":"Francesca","family":"Prignano","sequence":"additional","affiliation":[{"name":"Dermatology Unit, USL Toscana Centro, Ospedale P. Palagi, Viale Michelangelo, 41, 50125 Florence, Italy"},{"name":"Section of Dermatology, Department of Health Sciences, University of Florence, University of Florence Ospedale P. Palagi, Viale Michelangelo, 41, 50125 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,8,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1111\/jdv.18851","article-title":"Generalized Pustular Psoriasis: A Global Delphi Consensus on Clinical Course, Diagnosis, Treatment Goals and Disease Management","volume":"37","author":"Puig","year":"2023","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1007\/s40257-021-00664-x","article-title":"The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis","volume":"23","author":"Zheng","year":"2022","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"489","DOI":"10.23736\/S2784-8671.22.07415-1","article-title":"Pustular Psoriasis with a Focus on Generalized Pustular Psoriasis: Classification and Diagnostic Criteria. An Italian Expert Consensus","volume":"157","author":"Costanzo","year":"2023","journal-title":"Ital. J. Dermatol. Venereol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1007\/s13555-021-00493-0","article-title":"Unmet Medical Needs in the Treatment and Management of Generalized Pustular Psoriasis Flares: Evidence from a Survey of Corrona Registry Dermatologists","volume":"11","author":"Strober","year":"2021","journal-title":"Dermatol. Ther."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2711","DOI":"10.1080\/09546634.2022.2067819","article-title":"A Review of IL-36: An Emerging Therapeutic Target for Inflammatory Dermatoses","volume":"33","author":"Hwang","year":"2022","journal-title":"J. Dermatol. Treat."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1910","DOI":"10.1111\/jdv.19935","article-title":"The Role of Interleukin-36 in Health and Disease States","volume":"38","author":"Sugiura","year":"2024","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"155897","DOI":"10.1016\/j.cyto.2022.155897","article-title":"Role of IL-36 Cytokines in Psoriasis and Other Inflammatory Skin Conditions","volume":"156","author":"Sachen","year":"2022","journal-title":"Cytokine"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1681","DOI":"10.1007\/s40265-022-01801-4","article-title":"Spesolimab: First Approval","volume":"82","author":"Blair","year":"2022","journal-title":"Drugs"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1677","DOI":"10.2147\/CCID.S407812","article-title":"Use of Biological Therapies for the Management of Pustular Psoriasis: A New Era?","volume":"16","author":"Megna","year":"2023","journal-title":"Clin. Cosmet. Investig. Dermatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"EVIDoa2500272","DOI":"10.1056\/EVIDoa2500272","article-title":"Efficacy and Safety of Imsidolimab for Generalized Pustular Psoriasis","volume":"5","author":"Smieszek","year":"2026","journal-title":"NEJM Evid."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e72301","DOI":"10.1002\/hsr2.72301","article-title":"Janus Kinase Inhibitors for Treatment of Palmoplantar Pustulosis, Generalized Pustular Psoriasis, and Palmoplantar Pustular Psoriasis: A Systematic Review of the Literature","volume":"9","author":"Ansari","year":"2026","journal-title":"Health Sci. Rep."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1080\/1744666X.2019.1708193","article-title":"The Clinical, Humanistic, and Economic Burden of Generalized Pustular Psoriasis: A Structured Review","volume":"16","author":"Kharawala","year":"2020","journal-title":"Expert Rev. Clin. Immunol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"970","DOI":"10.1111\/bjd.20966","article-title":"Prevalence and Incidence of Generalized Pustular Psoriasis in Sweden: A Population-based Register Study","volume":"186","author":"Norlin","year":"2022","journal-title":"Br. J. Dermatol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1080\/03007995.2021.1894108","article-title":"Economic Burden of Generalized Pustular Psoriasis and Palmoplantar Pustulosis in the United States","volume":"37","author":"Hanna","year":"2021","journal-title":"Curr. Med. Res. Opin."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"adv44671","DOI":"10.2340\/actadv.v106.44671","article-title":"Economic Burden of Generalized Pustular Psoriasis: A Population-Based Register Study in Finland","volume":"106","author":"Teitsma","year":"2026","journal-title":"Acta Derm. Venereol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1093\/bjd\/ljae217","article-title":"Prevalence, Incidence, Mortality and Healthcare Resource Use for Generalized Pustular Psoriasis, Palmoplantar Pustulosis and Plaque Psoriasis in England: A Population-Based Cohort Study","volume":"191","author":"Frysz","year":"2024","journal-title":"Br. J. Dermatol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1111\/jdv.19901","article-title":"Generalized Pustular Psoriasis: A Nationwide Population-based Study Using the National Health Data System in France","volume":"38","author":"Viguier","year":"2024","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"T449","DOI":"10.1016\/j.ad.2024.03.013","article-title":"[Translated Article] Approach to the Epidemiology, Disease Management, and Current Challenges in the Management of Generalized Pustular Psoriasis Through a Survey Conducted Among Spanish Dermatologists","volume":"115","author":"Vilarrasa","year":"2024","journal-title":"Actas Dermo-Sifiliogr\u00e1ficas"},{"key":"ref_19","first-page":"589","article-title":"Epidemiology of Generalized Pustular Psoriasis in Germany: Analyzing Factors Influencing Prevalence Estimates from Claims Data","volume":"23","author":"Schultze","year":"2025","journal-title":"J. Dtsch. Dermatol. Ges."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1159\/000538072","article-title":"Epidemiology, Characteristics of Disease, and Unmet Needs of Patients with Generalized Pustular Psoriasis: A Large Italian Delphi Consensus","volume":"240","author":"Prignano","year":"2024","journal-title":"Dermatology"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.jdin.2021.12.001","article-title":"Generalized Pustular Psoriasis in Brazil: A Public Claims Database Study","volume":"6","author":"Duarte","year":"2022","journal-title":"JAAD Int."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e536","DOI":"10.1111\/jdv.19749","article-title":"Prevalence of Generalized Pustular Psoriasis in the United States Using the All of us Research Database","volume":"38","author":"Kam","year":"2024","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1111\/exd.14770","article-title":"Pustular Psoriasis in Malaysia: A Review of the Malaysian Psoriasis Registry 2007\u20132018","volume":"32","author":"Teoh","year":"2023","journal-title":"Exp. Dermatol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1093\/bjd\/ljad158","article-title":"Incidence and Prevalence of Generalized Pustular Psoriasis in Multiethnic Johor Bahru, Malaysia: A Population-Based Cohort Study Using Routinely Captured Electronic Health Records in the Teleprimary Care (TPC\u00ae) Clinical Information System from 2010 to 2020","volume":"189","author":"Choon","year":"2023","journal-title":"Br. J. Dermatol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1177\/24755303241257900","article-title":"Review of Generalized Pustular Psoriasis: Prevalence, Diagnosis, and Treatment Options","volume":"9","author":"Kozina","year":"2024","journal-title":"J. Psoriasis Psoriatic Arthritis"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1111\/jdv.18720","article-title":"Prevalence, Comorbidities and Mortality of Generalized Pustular Psoriasis: A Literature Review","volume":"37","author":"Prinz","year":"2023","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1016\/j.jaad.2022.09.049","article-title":"Comorbidities in Patients with Generalized Pustular Psoriasis: A Nationwide Population-Based Register Study","volume":"88","author":"Norlin","year":"2023","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"3379","DOI":"10.1007\/s13555-024-01306-w","article-title":"Characteristics of Patients with Generalized Pustular Psoriasis: A Report of the Polish Generalized Pustular Psoriasis Group","volume":"14","author":"Krajewski","year":"2024","journal-title":"Dermatol. Ther."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1111\/1346-8138.16217","article-title":"Western Japan Inflammatory Disease Research Group Clinical Characteristics of Japanese Pustular Psoriasis: A Multicenter Observational Study","volume":"49","author":"Ohata","year":"2022","journal-title":"J. Dermatol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1336","DOI":"10.1111\/1346-8138.15958","article-title":"Clinical Analysis of Generalized Pustular Psoriasis in Chinese Patients: A Retrospective Study of 110 Patients","volume":"48","author":"Zheng","year":"2021","journal-title":"J. Dermatol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"2345728","DOI":"10.1080\/09546634.2024.2345728","article-title":"Clinical Course and Disease Burden of Patients with Generalized Pustular Psoriasis in Portugal: A Multicenter Retrospective Cohort Study","volume":"35","author":"Torres","year":"2024","journal-title":"J. Dermatol. Treat."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1038\/s41423-020-0519-3","article-title":"\u201cAutoinflammatory Psoriasis\u201d\u2014Genetics and Biology of Pustular Psoriasis","volume":"18","author":"Uppala","year":"2021","journal-title":"Cell. Mol. Immunol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1007\/s40257-021-00655-y","article-title":"Pathophysiology of Generalized Pustular Psoriasis","volume":"23","author":"Marrakchi","year":"2022","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1056\/NEJMoa1013068","article-title":"Interleukin-36\u2013Receptor Antagonist Deficiency and Generalized Pustular Psoriasis","volume":"365","author":"Marrakchi","year":"2011","journal-title":"N. Engl. J. Med."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1194","DOI":"10.1111\/exd.14768","article-title":"Pathophysiology of Generalized Pustular Psoriasis","volume":"32","author":"Young","year":"2023","journal-title":"Exp. Dermatol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/j.jaci.2016.08.056","article-title":"IL-1 and IL-36 Are Dominant Cytokines in Generalized Pustular Psoriasis","volume":"140","author":"Johnston","year":"2017","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_37","doi-asserted-by":"crossref","unstructured":"Hawkes, J.E., Visvanathan, S., and Krueger, J.G. (2023). The Role of the Interleukin-36 Axis in Generalized Pustular Psoriasis: A Review of the Mechanism of Action of Spesolimab. Front. Immunol., 14.","DOI":"10.3389\/fimmu.2023.1292941"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1080\/1744666X.2022.2116003","article-title":"Generalized Pustular Psoriasis is a Disease Distinct from Psoriasis Vulgaris: Evidence and Expert Opinion","volume":"18","author":"Bachelez","year":"2022","journal-title":"Expert Rev. Clin. Immunol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"606","DOI":"10.1016\/j.pathol.2023.10.009","article-title":"Guttate Psoriasis Shares Histological Features with Pustular Psoriasis and Often Shows Neutrophil Exocytosis and Pustules","volume":"56","author":"Grolleau","year":"2024","journal-title":"Pathology"},{"key":"ref_40","doi-asserted-by":"crossref","unstructured":"Yang, S.-F., Lin, M.-H., Chou, P.-C., Hu, S.-K., Shih, S.-Y., Yu, H.-S., and Yu, S. (2023). Genetics of Generalized Pustular Psoriasis: Current Understanding and Implications for Future Therapeutics. Genes, 14.","DOI":"10.3390\/genes14061297"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"118","DOI":"10.3892\/ijmm.2021.4951","article-title":"An Update on Genetic Basis of Generalized Pustular Psoriasis (Review)","volume":"47","author":"Zhou","year":"2021","journal-title":"Int. J. Mol. Med."},{"key":"ref_42","doi-asserted-by":"crossref","unstructured":"Chen, B., Liu, Q., Dong, X., and Bai, Y. (2024). Biologics for Generalized Pustular Psoriasis: A Systematic Review and Single-Arm Meta-Analysis. Front. Immunol., 15.","DOI":"10.3389\/fimmu.2024.1462158"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1007\/s40257-021-00658-9","article-title":"Treatment Options and Goals for Patients with Generalized Pustular Psoriasis","volume":"23","author":"Krueger","year":"2022","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_44","doi-asserted-by":"crossref","unstructured":"Fischer, B., K\u00fcbelbeck, T., Kolb, A., Ringen, J., Waisman, A., Wittmann, M., Karbach, S., K\u00f6lsch, S.M., and Kramer, D. (2023). IL-17A-Driven Psoriasis is Critically Dependent on IL-36 Signaling. Front. Immunol., 14.","DOI":"10.3389\/fimmu.2023.1256133"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"5","DOI":"10.2340\/00015555-2808","article-title":"Highlighting Interleukin-36 Signalling in Plaque Psoriasis and Pustular Psoriasis","volume":"98","author":"Furue","year":"2018","journal-title":"Acta Derm. Venerol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1792","DOI":"10.1111\/jdv.14386","article-title":"European Consensus Statement on Phenotypes of Pustular Psoriasis","volume":"31","author":"Navarini","year":"2017","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1235","DOI":"10.1111\/1346-8138.14523","article-title":"Japanese Guidelines for the Management and Treatment of Generalized Pustular Psoriasis: The New Pathogenesis and Treatment of GPP","volume":"45","author":"Fujita","year":"2018","journal-title":"J. Dermatol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1007\/s13555-022-00881-0","article-title":"Generalized Pustular Psoriasis: A Review on Clinical Characteristics, Diagnosis, and Treatment","volume":"13","author":"Carrascosa","year":"2023","journal-title":"Dermatol. Ther."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1111\/exd.14794","article-title":"Diversity in the Clinical Presentation of Generalized Pustular Psoriasis (GPP): A Series of Case Vignettes from around the World","volume":"32","author":"Choon","year":"2023","journal-title":"Exp. Dermatol."},{"key":"ref_50","doi-asserted-by":"crossref","unstructured":"Bellinato, F., Gisondi, P., Marzano, A., Piaserico, S., De Simone, C., Damiani, G., Argenziano, G., Venturini, M., Dapavo, P., and Costanzo, A. (2023). Characteristics of Patients Experiencing a Flare of Generalized Pustular Psoriasis: A Multicenter Observational Study. Vaccines, 11.","DOI":"10.3390\/vaccines11040740"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1263","DOI":"10.1111\/exd.14776","article-title":"Clinical Profile of Patients with Acute Generalized Pustular Psoriasis with and without IL36RN Mutations in Multi-ethnic Johor Bahru, Malaysia","volume":"32","author":"Choon","year":"2023","journal-title":"Exp. Dermatol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1159\/000528753","article-title":"Different Clinical Features of Pediatric Generalized Pustular Psoriasis in Patients with or without IL36RN Variants","volume":"239","author":"Wang","year":"2023","journal-title":"Dermatology"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1177\/24755303251318976","article-title":"Management of Chronic Generalized Pustular Psoriasis: A Review and Expert Opinion","volume":"10","author":"Elewski","year":"2025","journal-title":"J. Psoriasis Psoriatic Arthritis\u00ae"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1159\/000529274","article-title":"Clinical Characteristics and Outcomes of Generalized Pustular Psoriasis Flares","volume":"239","author":"Choon","year":"2023","journal-title":"Dermatology"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"e14814","DOI":"10.1111\/dth.14814","article-title":"Drug Survival in the Treatment of Generalized Pustular Psoriasis: A Retrospective Multicenter Study","volume":"34","author":"Kromer","year":"2021","journal-title":"Dermatol. Ther."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"2261","DOI":"10.1007\/s13555-024-01228-7","article-title":"Clinical Characteristics and Disease Burden of Patients with Moderate-to-Severe Generalized Pustular Psoriasis Flares in Taiwan","volume":"14","author":"Lu","year":"2024","journal-title":"Dermatol. Ther."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"1142","DOI":"10.1001\/jamadermatol.2022.3142","article-title":"Understanding Flares in Patients with Generalized Pustular Psoriasis Documented in US Electronic Health Records","volume":"158","author":"Zema","year":"2022","journal-title":"JAMA Dermatol."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s40257-021-00654-z","article-title":"Clinical Course and Characteristics of Generalized Pustular Psoriasis","volume":"23","author":"Choon","year":"2022","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1684\/ejd.2021.4047","article-title":"Update on the Aetiology and Mechanisms of Generalized Pustular Psoriasis","volume":"31","author":"Wang","year":"2021","journal-title":"Eur. J. Dermatol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1007\/s40257-022-00698-9","article-title":"Generalized Pustular Psoriasis in Pregnancy: Current and Future Treatments","volume":"23","author":"Seishima","year":"2022","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"2091","DOI":"10.1007\/s13555-026-01691-4","article-title":"Real-World Burden of Generalized Pustular Psoriasis in a French Observational Study: Prevalence, Incidence, Healthcare Resource Utilization, Comorbidities, Treatment Use, and Mortality","volume":"16","author":"Viguier","year":"2026","journal-title":"Dermatol. Ther."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1007\/s00439-016-1750-y","article-title":"Mutations in IL36RN Are Associated with Geographic Tongue","volume":"136","author":"Liang","year":"2017","journal-title":"Hum. Genet."},{"key":"ref_63","doi-asserted-by":"crossref","unstructured":"Gershater, B., Bieber, K., Vorobyev, A., Ludwig, M.A., Zirpel, H., De Luca, D.A., Thaci, D., Kridin, K., and Ludwig, R.J. (2024). Differential Risks of Psoriatic Arthritis Development in Patients with Varied Psoriasis Manifestations: A Sex- and Ethnicity-Specific Analysis. Front. Med., 11.","DOI":"10.3389\/fmed.2024.1385491"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"828","DOI":"10.1093\/rheumatology\/kez358","article-title":"Interleukin-36 Family Dysregulation Drives Joint Inflammation and Therapy Response in Psoriatic Arthritis","volume":"59","author":"Boutet","year":"2020","journal-title":"Rheumatology"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1093\/cei\/uxac027","article-title":"IL-36\u03b3 in Enthesitis-Related Juvenile Idiopathic Arthritis and Its Association with Disease Activity","volume":"208","author":"Majumder","year":"2022","journal-title":"Clin. Exp. Immunol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/j.det.2024.01.001","article-title":"Generalized Pustular Psoriasis, Acute Generalized Exanthematous Pustulosis, and Other Pustular Reactions","volume":"42","author":"Oon","year":"2024","journal-title":"Dermatol. Clin."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1007\/s40257-023-00779-3","article-title":"Acute Generalized Exanthematous Pustulosis: Clinical Features, Differential Diagnosis, and Management","volume":"24","author":"Parisi","year":"2023","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1007\/s40257-021-00652-1","article-title":"Diagnosis of Generalized Pustular Psoriasis","volume":"23","author":"Fujita","year":"2022","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"e70227","DOI":"10.1111\/exd.70227","article-title":"Complications and Laboratory Test Findings Among Patients With Generalized Pustular Psoriasis: A Retrospective Chart Review Study","volume":"35","author":"Okuyama","year":"2026","journal-title":"Exp. Dermatol."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1111\/1346-8138.12863","article-title":"Clinical Features and Course of Generalized Pustular Psoriasis in Korea","volume":"42","author":"Jin","year":"2015","journal-title":"J. Dermatol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"1697","DOI":"10.1080\/03007995.2021.1951192","article-title":"Characteristics of Hospitalizations and Emergency Department Visits Due to Generalized Pustular Psoriasis in the United States","volume":"37","author":"Hanna","year":"2021","journal-title":"Curr. Med. Res. Opin."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1111\/ijd.12070","article-title":"Clinical Profile, Morbidity, and Outcome of Adult-onset Generalized Pustular Psoriasis: Analysis of 102 Cases Seen in a Tertiary Hospital in Johor, Malaysia","volume":"53","author":"Choon","year":"2014","journal-title":"Int. J. Dermatol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1219","DOI":"10.1111\/exd.14841","article-title":"A Narrative Review of the Socioeconomic Burden Associated with Generalised Pustular Psoriasis","volume":"32","author":"Zimmermann","year":"2023","journal-title":"Exp. Dermatol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1016\/j.jaad.2023.04.066","article-title":"Mortality in Generalized Pustular Psoriasis: A Population-Based National Register Study","volume":"89","author":"Ericson","year":"2023","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"1266","DOI":"10.1016\/j.jaad.2021.06.008","article-title":"Treatments and Outcomes of Generalized Pustular Psoriasis: A Cohort of 1516 Patients in a Nationwide Inpatient Database in Japan","volume":"86","author":"Miyachi","year":"2022","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_76","doi-asserted-by":"crossref","unstructured":"Teshima, R., Saito-Sasaki, N., and Sawada, Y. (2024). Generalized Pustular Psoriasis and Systemic Organ Dysfunctions. Int. J. Mol. Sci., 25.","DOI":"10.3390\/ijms25116270"},{"key":"ref_77","doi-asserted-by":"crossref","unstructured":"Gwillim, E.C., and Nichols, A.J. (2024). Spesolimab for Generalized Pustular Psoriasis: A Review of Two Key Clinical Trials Supporting Initial US Regulatory Approval. Front. Immunol., 15.","DOI":"10.3389\/fimmu.2024.1359481"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"2431","DOI":"10.1056\/NEJMoa2111563","article-title":"Trial of Spesolimab for Generalized Pustular Psoriasis","volume":"385","author":"Bachelez","year":"2021","journal-title":"N. Engl. J. Med."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1111\/1346-8138.16609","article-title":"Efficacy and Safety of Spesolimab in Asian Patients with a Generalized Pustular Psoriasis Flare: Results from the Randomized, Double-blind, Placebo-controlled EffisayilTM 1 Study","volume":"50","author":"Morita","year":"2023","journal-title":"J. Dermatol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1007\/s40265-023-01988-0","article-title":"Spesolimab for the Treatment of Generalized Pustular Psoriasis","volume":"84","author":"Bernardo","year":"2024","journal-title":"Drugs"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"1541","DOI":"10.1016\/S0140-6736(23)01378-8","article-title":"Efficacy and Safety of Subcutaneous Spesolimab for the Prevention of Generalised Pustular Psoriasis Flares (Effisayil 2): An International, Multicentre, Randomised, Placebo-Controlled Trial","volume":"402","author":"Morita","year":"2023","journal-title":"Lancet"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1402","DOI":"10.1016\/j.jaci.2021.09.035","article-title":"Pustular Psoriasis: Molecular Pathways and Effects of Spesolimab in Generalized Pustular Psoriasis","volume":"149","author":"Baum","year":"2022","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"1379","DOI":"10.1111\/1346-8138.17449","article-title":"Spesolimab, the First-in-class Anti-IL-36R Antibody: From Bench to Clinic","volume":"51","author":"Morita","year":"2024","journal-title":"J. Dermatol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/1346-8138.70122","article-title":"Real-World Safety Profile of Spesolimab in Generalized Pustular Psoriasis: Insights From Japan as Part of a Multinational Expanded Access Program (EAP)","volume":"53","author":"Morita","year":"2026","journal-title":"J. Dermatol."},{"key":"ref_85","doi-asserted-by":"crossref","unstructured":"Alzahrani, D., Hasanain, A., Alharthy, R., Aljefri, Y., Alghamdi, Y., Alshaikh, R., Alhijab, A., Neazy, F., Alosaimi, A., and Felemban, B. (2026). The Efficacy and Safety of Spesolimab in Patients with Generalized Pustular Psoriasis Flares: A Systematic Review and Meta-Analysis. Front. Med., 12.","DOI":"10.3389\/fmed.2025.1749320"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1111\/1346-8138.17074","article-title":"Deucravacitinib, an Oral, Selective, Allosteric Tyrosine Kinase 2 Inhibitor, in Japanese Patients with Moderate to Severe Plaque, Erythrodermic, or Generalized Pustular Psoriasis: Efficacy and Safety Results from an Open-label, Phase 3 Trial","volume":"51","author":"Imafuku","year":"2024","journal-title":"J. Dermatol."},{"key":"ref_87","doi-asserted-by":"crossref","unstructured":"Vila\u00e7a, J., Yilmaz, O., and Torres, T. (2024). New and Emerging Treatments for Generalized Pustular Psoriasis: Focus on IL-36 Receptor Inhibitors. Pharmaceutics, 16.","DOI":"10.3390\/pharmaceutics16070908"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1093\/bjd\/ljad083","article-title":"Imsidolimab, an Anti-Interleukin-36 Receptor Monoclonal Antibody, for the Treatment of Generalized Pustular Psoriasis: Results from the Phase II GALLOP Trial","volume":"189","author":"Warren","year":"2023","journal-title":"Br. J. Dermatol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1080\/09546634.2019.1682502","article-title":"Review of Treatments for Generalized Pustular Psoriasis","volume":"32","author":"Kearns","year":"2021","journal-title":"J. Dermatol. Treat."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1001\/jamadermatol.2016.0751","article-title":"Therapeutic Efficacy of Interleukin 12\/Interleukin 23 Blockade in Generalized Pustular Psoriasis Regardless of IL36RN Mutation Status","volume":"152","author":"Arakawa","year":"2016","journal-title":"JAMA Dermatol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"156353","DOI":"10.1016\/j.cyto.2023.156353","article-title":"Anti-Interleukin-17s for Successful Management of Pustular Psoriasis","volume":"171","author":"Guerra","year":"2023","journal-title":"Cytokine"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1931","DOI":"10.1111\/1346-8138.16164","article-title":"Epidemiology and Clinical Features of Pustular Psoriasis: A 15-year Retrospective Cohort","volume":"48","author":"Tosukhowong","year":"2021","journal-title":"J. Dermatol."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1016\/j.jaad.2023.04.043","article-title":"Comparative Efficacy of Biologics and Oral Agents in Palmoplantar Psoriasis and Palmoplantar Pustulosis: A Systematic Review and Network Meta-Analysis of Randomized Clinical Trials","volume":"89","author":"Spencer","year":"2023","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"6707","DOI":"10.2147\/JIR.S472338","article-title":"Comparative Efficacy and Safety of Ustekinumab and Secukinumab in the Treatment of Generalized Pustular Psoriasis: A 48-Week Retrospective Cohort Study with Genetic Background Analysis","volume":"17","author":"Ruan","year":"2024","journal-title":"J. Inflamm. Res."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1684\/ejd.2018.3245","article-title":"Association of IL36RN Mutations with Clinical Features, Therapeutic Response to Acitretin, and Frequency of Recurrence in Patients with Generalized Pustular Psoriasis","volume":"28","author":"Zhu","year":"2018","journal-title":"Eur. J. Dermatol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"1531","DOI":"10.1111\/jdv.19808","article-title":"Characteristics and Management of Generalized Pustular Psoriasis (GPP): Experience from the Central and Eastern Europe (CEE) GPP Expert Network","volume":"38","author":"Wolf","year":"2024","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_97","first-page":"778","article-title":"A Non-Interventional, Multicenter Study to Characterize the Socio-Demographics, Clinical Characteristics, and Management of Generalized Pustular Psoriasis Patients in Spain: IMPULSE Study","volume":"240","author":"Puig","year":"2024","journal-title":"Dermatology"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"e15593","DOI":"10.1111\/dth.15593","article-title":"Pustular Psoriasis of Pregnancy: Clinical and Genetic Characteristics in a Series of Eight Patients and Review of the Literature","volume":"35","author":"Ennouri","year":"2022","journal-title":"Dermatol. Ther."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"2334791","DOI":"10.1080\/09546634.2024.2334791","article-title":"Successful Treatment of Recalcitrant Generalized Pustular Psoriasis of Pregnancy with Spesolimab","volume":"35","author":"Yang","year":"2024","journal-title":"J. Dermatol. Treat."},{"key":"ref_100","first-page":"e59832","article-title":"Generalized Pustular Psoriasis of Pregnancy Successfully Treated With Certolizumab Pegol: A Case Report and Literature Review","volume":"16","author":"Pogorelov","year":"2024","journal-title":"Cureus"},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1917","DOI":"10.1007\/s13555-021-00612-x","article-title":"Pustular Psoriasis: A Narrative Review of Recent Developments in Pathophysiology and Therapeutic Options","volume":"11","author":"Menter","year":"2021","journal-title":"Dermatol. Ther."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1016\/j.jaad.2021.10.028","article-title":"Management of Pediatric Generalized Pustular Psoriasis Using Biologics: An Evidence-Based Review","volume":"87","author":"Sachdeva","year":"2022","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1002\/ped4.12395","article-title":"The Update of Treatment Strategies in Pediatrics with Generalized Pustular Psoriasis in China","volume":"7","author":"Chen","year":"2023","journal-title":"Pediatr. Investig."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"8199","DOI":"10.2147\/JIR.S485077","article-title":"Successful Treatment of Pediatric Generalized Pustular Psoriasis (GPP) with Spesolimab: 5 Case Reports and Evaluations of Circulating IL-36 Levels","volume":"17","author":"Chen","year":"2024","journal-title":"J. Inflamm. Res."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1080\/09546634.2017.1395798","article-title":"Efficacy and Safety of Acitretin Monotherapy in Children with Pustular Psoriasis: Results from 15 Cases and a Literature Review","volume":"29","author":"Chen","year":"2018","journal-title":"J. Dermatol. Treat."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1007\/s40257-021-00653-0","article-title":"Clinical Disease Measures in Generalized Pustular Psoriasis","volume":"23","author":"Burden","year":"2022","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1111\/jdv.18999","article-title":"Psychometric validation of the generalized pustular psoriasis physician global assessment (GPPGA) and generalized pustular psoriasis area and severity index (GPPASI)","volume":"37","author":"Burden","year":"2023","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"766","DOI":"10.1080\/00325481.2023.2285730","article-title":"Generalized Pustular Psoriasis: Practical Recommendations for Spanish Primary Care and Emergency Physicians","volume":"135","author":"Epelde","year":"2023","journal-title":"Postgrad. Med."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/15\/6087\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T09:51:15Z","timestamp":1785923475000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/15\/6087"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,5]]},"references-count":108,"journal-issue":{"issue":"15","published-online":{"date-parts":[[2026,8]]}},"alternative-id":["jcm15156087"],"URL":"https:\/\/doi.org\/10.3390\/jcm15156087","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,5]]}}}