Clinical Features, Treatment Effectiveness and Long-Term Outcomes in Children with Moderate-to-Severe Generalized Pustular Psoriasis: A Retrospective Cohort Study

Zhiqiang Cao,1,* Bin Peng,1,* Zonghao Li,2 Ruiqi Li,1 Ruitao Fan,1 Baibing Mi,2 Bingjie Li,1 Xiangjin Song,1 Jing Shi,1 Songmei Geng1 1Department of Dermatology, Northwest Hospital, The Second Hospital Affiliated of Xi’an Jiaotong University, Xi’an, Shaanxi, 710004, People’s Republic of China; 2Department of Epidemiology and Biostatistics, School of Public Health, Xi’an Jiaotong University Health Science Center, Xi’an, Shaanxi, 710061, People’s Republic of China*These authors contributed equally to this workCorrespondence: Songmei Geng, Department of Dermatology, Northwest Hospital, The Second Hospital Affiliated of Xi’an Jiaotong University, Xi’an, Shaanxi, 710004, People’s Republic of China, Tel +86 13060423612, Email [email protected]: Generalized pustular psoriasis (GPP) is a rare inflammatory skin disorder, frequently accompanied by systemic inflammatory manifestations. Pediatric GPP presents unique clinical features and requires tailored treatment approaches. However, real-world data on pediatric moderate-to-severe GPP remain limited.Objective: To describe the clinical profiles of pediatric moderate-to-severe GPP, evaluate the effectiveness of various treatments, and their impact on disease recurrence.Methods: This retrospective observational study enrolled pediatric moderate-to-severe GPP inpatients in our department from March 2017 to February 2024. Clinical characteristics, treatment regimens, and recurrence were collected and analyzed using electronic medical records and follow-up data.Results: 61 pediatric patients were included, with a male-to-female ratio of 1.54: 1, an average age of 10.6± 3.2 years. Concomitant symptoms were present in 93.4% of patients, including pruritus, fever, and skin pain. Common comorbidities included hypoproteinemia, anemia, and hyperlipidemia. Over 80% of patients exhibited elevated inflammatory markers, including IL-6, TNF-α. GPP with psoriasis vulgaris (PV) and without PV had similar clinical presentations. Regarding treatment outcomes, compared with traditional drugs, biologics significantly reduced both hospitalization time (6.0 days vs 8.0 days, P=0.002) and pustule clearance time (3.0 days vs 7.0 days, P=0.013), with a lower incidence of adverse events. Post-discharge follow-up data on annual flare indicated the biologics group was associated with fewer annual flares (0 vs 0.3 per person-year, P=0.052) and lower recurrence rate (21.1% vs 60.0%, P=0.034), compared with the traditional drugs group. Furthermore, serum proteomic analysis revealed significantly elevated IL-17 level and activation of the IL-17 signaling pathway in pediatric GPP compared with healthy controls, elucidating the mechanism underlying the high effectiveness of biologic-targeted therapies.Conclusion: For moderate-to-severe pediatric GPP, biologics exhibit faster effectiveness and better safety than traditional systemic drugs. Specifically, biologics can significantly reduce hospitalization time and pustule clearance time, and decrease disease recurrence.Keywords: children, clinical profile, generalized pustular psoriasis, moderate-to-severe, recurrence, treatment