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Recalcitrant paradoxical pustular psoriasis induced by infliximab:Two case reports 认领 引用 被引量：2
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作者 Ping Xia Yan-Hong Li +4 位作者 Zhong Liu Xu Zhang Qian Jiang Xiao-Yong Zhou Wei Su 《World Journal of Clinical Cases》 SCIE 2021年第15期3655-3661,共7页
BACKGROUND Paradoxical psoriasis induced by tumor necrosis factor alpha antagonists is a rare side effect of those drugs and has similarities with and differences from classical psoriasis in clinical and pathological ... BACKGROUND Paradoxical psoriasis induced by tumor necrosis factor alpha antagonists is a rare side effect of those drugs and has similarities with and differences from classical psoriasis in clinical and pathological characteristics.Treating severe paradoxical psoriasis is challenging because the reported cases are rare,with treatment experience being only anecdotal.CASE SUMMARY We report 2 cases of paradoxical psoriasis caused by infliximab.Both cases manifested with a significant number of pustular lesions and had protracted and complicated clinical courses.In case 1,secukinumab alone could not control the eruptions,but colchicine supplementation markedly decreased disease activity.In case 2 miscellaneous medications were administered,including the systemic drug acitretin,the immunosuppressive drug cyclosporine,and the biologic agent ustekinumab.However,multiple applications of those medications failed to prevent new lesions from occurring.Both cases showed moderate-to-high antinuclear antibody titers.CONCLUSION Based on these cases,moderate-to-high anti-nuclear antibody titer seems to be a risk factor for paradoxical psoriasis.In addition,extensive pustular presentation may be a negative prognostic indicator and may portend a protracted clinical course refractory to therapy. 展开更多
关键词 Pustular psoriasis Paradoxical Infliximab Secukinumab Ustekinumab Antinuclear antibody Case report
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Childhood pustular psoriasis--A case of a patient treated with traditional Chinese medicine 认领 引用
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作者 Liu Jun Zhang Juncha +4 位作者 Pan Lijia Zhang Xiaoqi Du Xiaoyi She Yanfen and Gao Weijuan 《World Journal of Integrated Traditional and Western Medicine》 2016年第2期19-21,共3页
Generalized pustulous psoriasis is characterized by the sudden onset of diffuse erythema, with a scattering of pustules, sometimes with fever and others symptoms. It is a relatively rare disease^1. The treatment of tr... Generalized pustulous psoriasis is characterized by the sudden onset of diffuse erythema, with a scattering of pustules, sometimes with fever and others symptoms. It is a relatively rare disease^1. The treatment of traditional western medicine have various adverse drug reactions and easy to recur. Meanwhile, this report describes a 12-year-old female patient of generalized pustulous psoriasis with a satisfactory effect of traditional Chinese medicine. 展开更多
关键词 Generalized pustular psoriasis Traditional Chinese medicine Satisfactory effects
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Clinical differentiation between acute generalized exanthematous pustulosis and generalized pustular psoriasis:A multi-center study 认领 引用
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作者 Shuxian Guo Xuming Wang +5 位作者 Wenhao Cheng Zuoyao Qi Yao Sun Linlin Ma Fanxiang Wang Guan Jiang 《Chinese Medical Journal》 SCIE CAS CSCD 2026年第8期1257-1259,共3页
To the Editor:Acute generalized exanthematous pustulosis(AGEP)and generalized pustular psoriasis(GPP)both present with widespread sterile pustules,sometimes forming large pustular lakes,and share overlapping clinical ... To the Editor:Acute generalized exanthematous pustulosis(AGEP)and generalized pustular psoriasis(GPP)both present with widespread sterile pustules,sometimes forming large pustular lakes,and share overlapping clinical and histological features.[1,2]However,they differ significantly in cause and prevalence.AGEP is a rare,severe drug-induced reaction mediated by T cells,affecting 1–5 people per million.In contrast,GPP is a rare but potentially life-threatening form of psoriasis,observed in about 14 individuals per million.Approximately 50%of GPP patients have pre-existing psoriasis vulgaris(PsV),and many endure delayed diagnosis due to clinical similarity,leading to prolonged symptoms and impaired quality of life.Although genetic analyses reveal minimal molecular distinctions between these entities(PDK4 and ZBTB16),[3]their distinct triggers and disease courses make accurate diagnosis essential for proper treatment. 展开更多
关键词 sterile pustulessometimes generalized pustular psoriasis gpp both pustular lakesand Drug Induced Reaction Prevalence Psoriasis Vulgaris Acute Generalized Exanthematous Pustulosis Generalized Pustular Psoriasis
Clinical and genetic characteristics of generalized pustular psoriasis:A retrospective study based on a registry database 认领 引用
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作者 Yusha Chen Yue Zhang +8 位作者 Ziyu Zhou Jia Geng Zhongtao Li Deyu Song Yiyi Wang Sheng Wang Jingyi Li Fengxiao Bu Wei Li 《Chinese Medical Journal》 SCIE CAS CSCD 2026年第4期631-632,共2页
To the Editor:Generalized pustular psoriasis(GPP)is a rare,severe inflammatory dermatosis that exhibits significant clinical and genetic heterogeneity which is characterized by primary acute widespread sterile pustule... To the Editor:Generalized pustular psoriasis(GPP)is a rare,severe inflammatory dermatosis that exhibits significant clinical and genetic heterogeneity which is characterized by primary acute widespread sterile pustules or subacute annular variant arising on erythematous skin background.Due to its rarity and complexity,the phenotype and genotype remain under-documented.[1,2]This study aimed to investigate the clinical and genetic profiles of GPP in a large single-center cohort based on flare frequency,IL36RN mutations,psoriasis vulgaris(PsV)history,and age of GPP onset.This study adhered to the Declaration of Helsinki and was approved by the Ethics Committee of Sichuan University West China Hospital(No.2024-1473).Informed consent was obtained orally via telephone or in writing during face-to-face inquiries. 展开更多
关键词 clinical genetic profiles primary acute widespread sterile pustules registry database generalized pustular psoriasis clinical characteristics pustular psoriasis gpp genetic characteristics flare frequency
Generalized pustular psoriasis:Advances in pathogenesis,diagnosis,and treatment 认领 引用
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作者 Zhen Li Hong Liu +1 位作者 Yonghu Sun Furen Zhang 《Chinese Medical Journal》 SCIE CAS CSCD 2026年第12期1805-1807,共3页
Generalized pustular psoriasis(GPP)is a rare but severe,potentially life-threatening condition characterized by widespread rashes and sterile pustule eruptions on nonacral skin.Flares often include systemic symptoms,s... Generalized pustular psoriasis(GPP)is a rare but severe,potentially life-threatening condition characterized by widespread rashes and sterile pustule eruptions on nonacral skin.Flares often include systemic symptoms,such as high fever and malaise,which may lead to sepsis and multiorgan failure,with mortality rates between 4%and 24%.Reported prevalence rates differ widely,ranging from 1.76 to 198 cases per million people.[1]A variety of proinflammatory cytokines,particularly interleukin(IL)-36,are central to GPP pathogenesis.Its rapid onset and recurrent flares present significant management challenges.However,recent years have seen substantial progress in understanding GPP pathogenesis,development of targeted biological therapies,and clinical guideline updates,offering new hope for patients. 展开更多
关键词 proinflammatory cytokinesparticularly pathogenesis treatment proinflammatory cytokines generalized pustular psoriasis systemic symptomssuch diagnosis generalized pustular psoriasis gpp
Hospitalized generalized pustular psoriasis vs. acute generalized exanthematous pustulosis: A comprehensive analysis of clinical manifestations and treatment outcomes 认领 引用
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作者 Kun Hu Jingge Xiao +5 位作者 Honghong Chang Hong Zhang Qianzi Liu Jing Zhang Yehong Kuang Wu Zhu 《Chinese Medical Journal》 SCIE CAS CSCD 2026年第11期1715-1717,共3页
To the Editor:Generalized pustular psoriasis(GPP)is a rare,chronic,and life-threatening condition characterized by widespread eruption of sterile pustules.[1,2]The mortality rate of GPP is 39.2 per 1000 person-years,w... To the Editor:Generalized pustular psoriasis(GPP)is a rare,chronic,and life-threatening condition characterized by widespread eruption of sterile pustules.[1,2]The mortality rate of GPP is 39.2 per 1000 person-years,with over half of the patients requiring hospitalization.[3]Another non-follicular pustular disease,acute generalized exanthematous pustulosis(AGEP),is currently defined as a rare,acute,and severe cutaneous adverse reaction.[4]The confusion between GPP and AGEP remains a clinical challenge,highlighting the urgency for additional diagnostic markers to distinguish between these two conditions.Therefore,we aim to provide analytical insights that facilitate the diagnosis of these two distinct dermatological entities. 展开更多
关键词 pustular psoriasis gpp Acute Generalized Exanthematous Pustulosis Clinical Manifestations Diagnostic Markers generalized exanthematous pustulosis agep widespread eruption sterile pustules Treatment Outcomes Generalized Pustular Psoriasis
Systemic Immune Inflammatory Index Is Associated with Pustular Psoriasis: A Single Center Retrospective Study 认领 引用
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作者 Salah Hassan Ibrahim Mengqi Guan Shanshan Li 《Journal of Cosmetics, Dermatological Sciences and Applications》 2022年第4期174-186,共13页
Introduction: Psoriasis is a chronic multi-systemic inflammatory skin disease that presents with erythema, thickness, and scaling of the skin. Genetic and environmental factors are associated with its etiology. Recent... Introduction: Psoriasis is a chronic multi-systemic inflammatory skin disease that presents with erythema, thickness, and scaling of the skin. Genetic and environmental factors are associated with its etiology. Recently systemic immune inflammatory index, has been proposed as a biomarker for prognosis and severity prediction. Although it has been studied in psoriasis in general, no study exists for its association with the individual types of psoriasis. This study thus aimed to determine its association with clinical characteristics of psoriasis subtypes. Materials and Methods: Data were retrospectively retrieved from the hospital electronic medical database from January 2020 to August 2022. Only patients with CBC results were included. Clinical data retrieved were: Patients’ age, gender, type of psoriasis diagnosed, body mass index, duration of the disease, family history of psoriasis, history of smoking, diabetes, and hypertension records. Laboratory data retrieved were: Complete blood count (CBC), C-reactive protein, Immunoglobulin E (IgE), Total cholesterol, Triglycerides and Low-density lipoprotein cholesterol. Data were analyzed in SPSS and GraphPad prism. Results: The study enrolled 85 patients with psoriasis;56.47% males, and 43.53% females. 7.6% had psoriasis for less than 10 years, while 42.4% had the disease for more than 10 years. Psoriasis vulgaris was the most common diagnosis, 41.2%, followed by p. pustular, 30.6% and then p. erythroderma 28.2%. Mean age ± SD of the p. vulgaris, p. pustular and p. erythroderma patients were 47.3 ± 15.3;45.3 ± 14.6, and 57.1 ± 11.7 respectively. SII was significantly higher in p. pustular than the rest, (p Conclusion: In summary, systemic immune inflammatory index (SII) was significantly higher in psoriasis pustular than other subtypes of psoriasis, and had an association with hypertension in psoriasis pustular patients. These findings suggest a possible association between SII and psoriasis pustular that should be investigated in an independent study. 展开更多
关键词 Systemic Immune Inflammatory Index Psoriasis Pustular
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Neutrophils in generalized pustular psoriasis:Key players in disease progression 认领 引用
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作者 Xin Zhang Yuting Xia +2 位作者 Haoyu Li Juan Tao Jing Yang 《Chinese Medical Journal》 SCIE CAS CSCD 2026年第13期2027-2029,共3页
To the Editor:Generalized pustular psoriasis(GPP)is a rare and highly heterogeneous autoinflammatory skin disease characterized by neutrophil infiltration of the epidermis.Recent advances have highlighted neutrophils ... To the Editor:Generalized pustular psoriasis(GPP)is a rare and highly heterogeneous autoinflammatory skin disease characterized by neutrophil infiltration of the epidermis.Recent advances have highlighted neutrophils as central drivers of GPP pathogenesis through multifaceted mechanisms.The pathogenesis of GPP involves a synergistic interplay between genetic predisposition and environmental triggers.Key genetic drivers of this disorder include mutations in the IL36RN,CARD14,TNIP1,BTN3A3,AP1S3,and SERPINA3 genes,which promote the onset of GPP via the dysregulation of interleukin(IL)-36 production.[1,2]Environmental triggers encompass infection,hypocalcemia,medication regimens(e.g.,abrupt glucocorticoid withdrawal),and pregnancy.[1]A notable example by Sugiura et al[3]highlights this interaction:monozygotic twins with compound heterozygous IL36RN mutations developed GPP after amoxicillin exposure.This suggested thatβ-lactam antibiotics(e.g.,amoxicillin)may synergize with bacterial infections to activate cutaneous inflammatory cells,thereby triggering excessive IL-36 production.In genetically predisposed individuals with IL36RN mutations,hypersensitivity to IL-36 signaling leads to uncontrolled neutrophil recruitment and systemic pustular eruptions. 展开更多
关键词 neutrophil infiltration pustular psoriasis gpp environmental triggers serpina gen neutrophils genetic predisposition pathogenesis IL
Annular pustular psoriasis:A heterogeneous subtype of generalized pustular psoriasis 认领 引用
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作者 Xiaoyan Wu Changxu Han +1 位作者 Zhengfeng Li Zhenying Zhang 《Chinese Medical Journal》 SCIE CAS CSCD 2025年第13期1630-1632,共3页
To the Editor:Annular pustular psoriasis(APP),a rare variant of generalized pustular psoriasis(GPP),manifests as recurrent,ring-shaped erythematous lesions adorned with small,sterile pustules,following a subacute traj... To the Editor:Annular pustular psoriasis(APP),a rare variant of generalized pustular psoriasis(GPP),manifests as recurrent,ring-shaped erythematous lesions adorned with small,sterile pustules,following a subacute trajectory.The recurrent nature of APP substantially diminishes patient quality of life.Owing to its infrequency,unified treatment protocols remain elusive. 展开更多
关键词 Ring Shaped Lesions generalized pustular psoriasis gpp manifests pustular psoriasis app Annular Pustular Psoriasis Small Sterile Pustules Recurrent Subacute Trajectory Erythematous
Current status of generalized pustular psoriasis:Findings from a multicenter hospital-based survey of 127 Chinese patients 认领 引用 被引量：2
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作者 Haimeng Wang Jiaming Xu +28 位作者 Xiaoling Yu Siyu Hao Xueqin Chen Bin Peng Xiaona Li Ping Wang Chaoyang Miao Jinzhu Guo Qingjie Hu Zhonglan Su Sheng Wang Chen Yu Qingmiao Sun Minkuo Zhang Bin Yang Yuzhen Li Zhiqiang Song Songmei Geng Aijun Chen Zigang Xu Chunlei Zhang Qianjin Lu Yan Lu Xian Jiang Gang Wang Hong Fang Qing Sun Jie Liu Hongzhong Jin 《Chinese Medical Journal》 SCIE CAS CSCD 2025年第8期953-961,共9页
Background:Generalized pustular psoriasis(GPP),a rare and recurrent autoinflammatory disease,imposes a substantial burden on patients and society.Awareness of GPP in China remains limited.Methods:This cross-sectional ... Background:Generalized pustular psoriasis(GPP),a rare and recurrent autoinflammatory disease,imposes a substantial burden on patients and society.Awareness of GPP in China remains limited.Methods:This cross-sectional survey,conducted between September 2021 and May 2023 across 14 hospitals in China,included GPP patients of all ages and disease phases.Data collected encompassed demographics,clinical characteristics,economic impact,disease severity,quality of life,and treatment-related complications.Risk factors for GPP recurrence were analyzed.Results:Among 127 patients(female/male ratio=1.35:1),the mean age of disease onset was 25 years(1st quartile[Q1]–3rd quartile[Q3]:11–44 years);29.2%had experienced GPP for more than 10 years.Recurrence occurred in 75.6%of patients,and nearly half reported no identifiable triggers.Younger age at disease onset(P=0.021)and transitioning to plaque psoriasis(P=0.022)were associated with higher recurrence rates.The median diagnostic delay was 8 months(Q1–Q3:2–41 months),and 32.3%of patients reported misdiagnoses.Comorbidities were present in 53.5%of patients,whereas 51.1%experienced systemic complications during treatment.Depression and anxiety affected 84.5%and 95.6%of patients,respectively.During GPP flares,the median Dermatology Life Quality Index score was 19.0(Q1–Q3:13.0–23.5).This score showed significant differences between patients with and without systemic symptoms;it demonstrated correlations with both depression and anxiety scores.Treatment costs caused financial hardship in 55.9%of patients,underscoring the burden associated with GPP.Conclusions:The substantial disease and economic burdens among Chinese GPP patients warrant increased attention.Patients with early onset disease and those transitioning to plaque psoriasis require targeted interventions to mitigate the high recurrence risk. 展开更多
关键词 Generalized pustular psoriasis Burden Misdiagnosis Recurrence Symptoms Comorbidities Treatments
Etanercept:A viable treatment option for young children with generalized pustular psoriasis 认领 引用 被引量：2
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作者 Yunliu Chen Zhaoyang Wang +2 位作者 Chaoyang Miao Zigang Xu Xin Xiang 《Pediatric Investigation》 CAS CSCD 2024年第4期295-298,共4页
Generalized pustular psoriasis(GPP)is a severe inflammatory cutaneous disease characterized by widespread pustules,edema,erythema,fever,and systemic inflammation.Chinese data indicate that the prevalence and incidence... Generalized pustular psoriasis(GPP)is a severe inflammatory cutaneous disease characterized by widespread pustules,edema,erythema,fever,and systemic inflammation.Chinese data indicate that the prevalence and incidence of GPP follow a bimodal age distribution,with peaks in the 0–3 year age group and the 30–39 year age group.1 In the 0–3 year age group,the prevalence was 0.927 and the incidence rate was 0.742 per 100000 population-years.1 Interleukin(IL)-36 plays an important role in GPP by activating nuclear factor-κB(NF-κB)and mitogen-activated protein kinase(MAPK)signal pathways.2 Chemokines(CXCL8,CXCL1,CXCL2,etc.),cytokines(IL-1β,tumor necrosis factor TNF-α,IL-6,IL-23,IL-17,etc.),and activated cells(e.g.,keratinocyte,neutrophils,dendritic cells,etc.)are also involved.3 Acitretin,cyclosporine,methotrexate,and etanercept were recommended as first-line treatments for children with GPP in 2012 by the American National Psoriasis Foundation.4 However,recent findings suggest that biological agents targeting IL-36,TNF-α,IL-17,IL-23,or their receptors might be more promising options than conventional drugs.5 Clinical trials and case series have also demonstrated the superiority of biological agents in adult and older pediatrics with GPP,including spesolimab,etanercept,adalimumab,secukinumab,brodalumab,and others.6 However,these biological agents,have only been approved by the FDA for children aged 4 years or older,and there is still limited data on their use in GPP patients under 4 years old. 展开更多
关键词 severe inflammatory cutaneous disease generalized pustular psoriasis TNF children etanercept generalized pustular psoriasis gpp IL activating nuclear f
“Autoinflammatory psoriasis”-genetics and biology of pustular psoriasis 认领 引用 被引量：24
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作者 Ranjitha Uppala Lam C.Tsoi +6 位作者 Paul W.Harms Bo Wang Allison C.Billi Emanual Maverakis J.Michelle Kahlenberg Nicole L.Ward Johann E.Gudjonsson 《Cellular & Molecular Immunology》 SCIE CAS CSCD 2021年第2期307-317,共11页
Psoriasis is a chronic inflammatory skin condition that has a fairly wide range of clinical presentations.Plaque psoriasis,which is the most common manifestation of psoriasis,is located on one end of the spectrum,domi... Psoriasis is a chronic inflammatory skin condition that has a fairly wide range of clinical presentations.Plaque psoriasis,which is the most common manifestation of psoriasis,is located on one end of the spectrum,dominated by adaptive immune responses,whereas the rarer pustular psoriasis lies on the opposite end,dominated by innate and autoinflammatory immune responses.In recent years,genetic studies have identified six genetic variants that predispose to pustular psoriasis,and these have highlighted the role of IL-36 cytokines as central to pustular psoriasis pathogenesis.In this review,we discuss the presentation and clinical subtypes of pustular psoriasis,contribution of genetic predisposing variants,critical role of the IL-36 family of cytokines in disease pathophysiology,and treatment perspectives for pustular psoriasis.We further outline the application of appropriate mouse models for the study of pustular psoriasis and address the outstanding questions and issues related to our understanding of the mechanisms involved in pustular psoriasis. 展开更多
关键词 Pustular psoriasis Genetics IL-36 Autoinflammation Clinical features Histology
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Tumor Necrosis Factor-alpha Induced Protein 3 Interacting Protein I Gene Polymorphisms and Pustular Psoriasis in Chinese Han Population 认领 引用 被引量：11
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作者 Jian-Wen Han Yong Wang +4 位作者 Chulu Alateng Hong-Bin Li Yun-Hua Bai Xin-Xiang Lyu Rina Wu 《Chinese Medical Journal》 SCIE CAS CSCD 2016年第13期1519-1524,共6页
Background： Psoriasis is a common immune-mediated inflammatory dermatosis. Generalized pustular psoriasis （GPP） is the severe and rare type of psoriasis. The association between tumor necrosis factor-alpha induced ... Background： Psoriasis is a common immune-mediated inflammatory dermatosis. Generalized pustular psoriasis （GPP） is the severe and rare type of psoriasis. The association between tumor necrosis factor-alpha induced protein 3 interacting protein 1 （TNIP1） gene and psoriasis was confirmed in people with multiple ethnicities. This study was to investigate the association between TNIP1 gene polymorphisms and pustular psoriasis in Chinese Hart population. Methods： Seventy-three patients with GPP, 67 patients with palmoplantar pustulosis （PPP）, and 476 healthy controls were collected from Chinese Hart population. Six single nucleotide polymorphisms （SNPs） of the TNIP1 gene, namely rs3805435, rs3792798, rs3792797, rs869976, rs 17728338, and rs999011 were genotyped by using polymerase chain reaction-ligase detection reaction. Statistical analyses were performed using the PLINK 1.07 package. Allele frequencies and genotyping frequencies for six SNPs were compared by using Chi-square test, odd ratio （OR） （including 95% confidence interval） were calculated. The haplotype analysis was conducted by Haploview software. Results： The frequencies of alleles of five SNPs were significantly different between the GPP group and the control group （P ≤ 7.22 × 10^-3）, especially in the GPP patients without psoriasis vulgaris （PsV）. In the haplotype analysis, the most significantly different haplotype was H4： ACGAAC, with 13.1% frequency in the GPP group but only 3.4% in the control group （OR = 4.16, P = 4.459 × 10^-7）. However, no significant difference in the allele frequencies was found between the PPP group and control group for each of the six SNPs （P 〉 0.05）. Conclusions： Polymorphisms in TNIP1 are associated with GPP in Chinese Han population. However, no association with PPP was found. These findings suggest that TNIPI might be a susceptibility gene for GPE 展开更多
关键词 Association Pustular Psoriasis Single Nucleotide Polymorphism Tumor Necrosis Factor-alpha Induced Protein 3Interacting Protein 1
The update of treatment strategies in pediatrics with generalized pustular psoriasis in China 认领 引用 被引量：2
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作者 Yunliu Chen Xin Xiang +2 位作者 Zhaoyang Wang Chaoyang Miao Zigang Xu 《Pediatric Investigation》 CAS CSCD 2023年第3期191-198,共8页
Generalized pustular psoriasis(GPP)is a severe subtype of psoriasis,commonly combined with systemic inflammation.Gene mutations have been found to be associated with GPP and vary by ethnicity.Systemic treatments are u... Generalized pustular psoriasis(GPP)is a severe subtype of psoriasis,commonly combined with systemic inflammation.Gene mutations have been found to be associated with GPP and vary by ethnicity.Systemic treatments are usually required for the severity and potential complications of GPP.However,there is no common consensus in China,especially among pediatric patients,whose data are scarce.Acitretin,methotrexate,and cyclosporine are widely used in pediatrics with GPP,while the adverse effects should be highlighted.The emergence of different biological agents brings us into a new era.This article discusses the genetic background of Chinese patients and demonstrates the evidence of treatment in pediatrics with GPP. 展开更多
关键词 Generalized pustular psoriasis Pediatrics Treatment
以骨痛为主要症状的老年女性SAPHO综合征误诊分析 认领 引用
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作者 于贤 曾少静 +4 位作者 孟娇娇 周悦 陈树春 任路平 吕秀芹 《临床误诊误治》 CAS 2026年第4期8-12,共5页
目的探讨以骨痛为主要症状的老年女性滑膜炎-痤疮-脓疱病-骨肥厚-骨炎(SAPHO)综合征合并糖尿病患者的临床特点。方法回顾性分析2023年收治的1例曾误诊为其他疾病的SAPHO综合征老年女性患者的临床资料。结果本例老年患者主因全身疼痛14年... 目的探讨以骨痛为主要症状的老年女性滑膜炎-痤疮-脓疱病-骨肥厚-骨炎(SAPHO)综合征合并糖尿病患者的临床特点。方法回顾性分析2023年收治的1例曾误诊为其他疾病的SAPHO综合征老年女性患者的临床资料。结果本例老年患者主因全身疼痛14年,加重1个月入院,曾多次被误诊为颈椎病、腰椎病、骨质疏松、骨关节炎、痛风、糖尿病周围神经病等,予以对症治疗效果不佳。此次入院后综合患者骨痛、脓疱疹等临床表现,结合全身骨显像提示双侧第一胸肋关节、胸骨角显像剂浓聚影,确诊为SAPHO综合征。误诊时间14年。确诊后给予调节免疫治疗后病情明显好转。随访过程中,患者疼痛相关症状较初始状态显著改善。结论SAPHO综合征病因多种多样,易造成漏诊和误诊,临床上需要仔细甄别;该病临床表现缺乏特异性,早期症状常不典型,加强对该病的认识、提高警惕性,有利于早期确诊,尽早治疗。 展开更多
关键词 SAPHO综合征 误诊 颈椎病 骨痛 骨质疏松 脓疱疹 糖尿病周围神经病
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古塞奇尤单抗成功治疗阿达木单抗诱发的泛发性脓疱型银屑病一例并文献复习 认领 引用
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作者 白帆 于万龙 +2 位作者 魏禹铭 许雅波 雷明君 《中国麻风皮肤病杂志》 2026年第2期97-100,共4页
生物制剂已经广泛应用于皮肤病的治疗,但在治疗过程中可能会诱发其他疾病或导致原有疾病加重等不良反应,称为矛盾性反应。本文报道一例斑块状银屑病患者使用阿达木单抗治疗,出现泛发性脓疱型银屑病的矛盾性反应,更换为古塞奇尤单抗治疗... 生物制剂已经广泛应用于皮肤病的治疗,但在治疗过程中可能会诱发其他疾病或导致原有疾病加重等不良反应,称为矛盾性反应。本文报道一例斑块状银屑病患者使用阿达木单抗治疗,出现泛发性脓疱型银屑病的矛盾性反应,更换为古塞奇尤单抗治疗。治疗第12周时患者皮损已完全消退,无不良反应发生,目前仍在治疗随访中。 展开更多
关键词 古塞奇尤单抗 阿达木单抗 泛发性脓疱型银屑病 矛盾性反应
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Understanding the Pathogenesis of Generalized Pustular Psoriasis Based on Molecular Genetics and Immunopathology 认领 引用 被引量：1
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作者 An-Qi Zhao Ming Li 《International Journal of Dermatology and Venereology》 2022年第4期199-206,共8页
Generalized pustular psoriasis(GPP)is a rare and life-threatening autoinflammatory skin disease characterized by recurrent and sudden episodes of widespread rashes with scattered sterile pustules.Clinical and genetic ... Generalized pustular psoriasis(GPP)is a rare and life-threatening autoinflammatory skin disease characterized by recurrent and sudden episodes of widespread rashes with scattered sterile pustules.Clinical and genetic evidence indicates that the pathogenesis of GPP both overlaps and is separate from psoriasis vulgaris(PV).Interleukin(IL)-23/IL-17 immune pathway is well known to play a critical role in the immunopathogenesis of PV,while the inflammation of GPP is more inclined to involve the innate immune response via the IL-1/IL-36-chemokine pathway.Mutations in IL36RN,CARD13,AP1S3,MPO,TNIP1,SERPINA3,and SERPINA1 have been shown to be associated with GPP,among which loss-of-function mutation in IL36RN is the dominant mutation with the highest prevalence.Recent studies have shown that interaction of the IL-36 pathway and the IL-23/IL-17 axis underlies the immunological disturbances of GPP,indicating that innate and adaptive immune responses intertwine in the pathogenesis of GPP.With this deeper understanding of the pathogenesis of GPP,treatment by biologics targeting the IL-1/IL-36 pathway appears to be promising.IL-1 inhibitors,anakinra,canakinumab,and gevokizumab have reportedly been effective in some cases.Spesolimab and imsidolimab,which are antibodies to the IL-36 receptor,are undergoing investigation in a phase II trial and showing promising results.In the present review,we illustrate the current understanding of the pathogenesis of GPP based on recent updates on the molecular genetics and immunopathology of GPP and review recent clinical trials and case reports of novel biologics in the treatment of GPP. 展开更多
关键词 autoinflammation generalized pustular psoriasis GPP genetic mechanisms IL-36
佩索利单抗治疗泛发性脓疱型银屑病:31例病例汇总的真实世界数据分析 认领 引用
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作者 刘润秋 蒋燕南 +3 位作者 刘龙丹 孙晓明 陈旭东 秦萍萍 《皮肤性病诊疗学杂志》 2026年第7期493-497,共5页
目的评价佩索利单抗在泛发性脓疱型银屑病(GPP)患者真实世界临床实践中的疗效与安全性特征。方法采用回顾性病例分析联合系统性文献综述的方法。收集本中心收治的5例接受佩索利单抗治疗的GPP患者资料,并系统检索中英文数据库,纳入相关... 目的评价佩索利单抗在泛发性脓疱型银屑病(GPP)患者真实世界临床实践中的疗效与安全性特征。方法采用回顾性病例分析联合系统性文献综述的方法。收集本中心收治的5例接受佩索利单抗治疗的GPP患者资料,并系统检索中英文数据库,纳入相关中国病例报告,分析患者数据。观察指标包括脓疱消退时间、GPP医师整体评估(GPPGA)评分、GPP面积与严重程度指数(GPPASI)评分及不良事件。结果最终汇总分析31例GPP患者,皮肤脓疱完全消退的中位时间为2 d,45.16%(14/31)的患者在48 h内消退。所有患者第7天GPPGA和GPPASI评分均较基线显著下降。5例妊娠期患者及数例高龄伴严重合并症者均治疗成功且结局良好。8例患者采用佩索利单抗联合糖皮质激素方案。安全性方面,总体耐受性良好,但32.26%(10/31)的患者出现原有银屑病皮损红斑加重或新发红斑红斑扩大。结论在临床实践中,佩索利单抗对中国GPP患者,包括妊娠期及复杂共病患者,展现出快速、强效的诱导缓解作用。联合糖皮质激素是处理急重症的常用策略。需高度重视其可能引发原有皮损加重的特征性反应,并制定全面的急性期与长期管理计划。 展开更多
关键词 泛发性脓疱型银屑病 佩索利单抗 真实世界研究 疗效 安全性
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150例泛发性脓疱型银屑病患者的临床表征回顾性分析 认领 引用
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作者 余晓玲 刘若昀 +1 位作者 石燕强 杨斌 《皮肤性病诊疗学杂志》 2026年第3期170-178,共9页
目的分析泛发性脓疱型银屑病(GPP)患者的临床表征,为GPP患者的健康管理提供更多理论支持。方法回顾性分析2017年7月至2023年7月期间就诊于南方医科大学皮肤病医院的150例GPP患者的资料,根据发病年龄、性别、GPP是否伴发寻常型银屑病(PV... 目的分析泛发性脓疱型银屑病(GPP)患者的临床表征,为GPP患者的健康管理提供更多理论支持。方法回顾性分析2017年7月至2023年7月期间就诊于南方医科大学皮肤病医院的150例GPP患者的资料,根据发病年龄、性别、GPP是否伴发寻常型银屑病(PV)进行分组,并探寻不同分组GPP患者的发病季节和诱因、复发频率、伴发疾病及治疗效果等临床表征的差异。结果150例GPP患者平均发病年龄(32.83±21.65)岁。单纯GPP组患者的发病年龄小于GPP+PV组患者[(28.30±19.27)岁比(40.87±23.43)岁,t=-3.51,P18岁患者的复发诱因多为治疗不当(39.5%),而发病年龄≤18岁患者的复发诱因多为上呼吸道感染(40.0%),部分女性患者复发与妊娠、月经期等特殊生理状态相关。发病年龄≤18岁的患者出现复发的比例(91.3%)较发病年龄>18岁患者(71.7%)高(χ2=7.39,P<0.05)。女性1年内复发3~4次的概率高于男性(χ2=4.20,P<0.05);单纯GPP组患者更易复发,其1年内复发1~2次、1~4次的概率显著高于GPP+PV组患者(均P<0.05))。男性患者出现甲损害、中热的概率均高于女性(P<0.05),但出现高热的概率小于女性(χ2=3.90,P<0.05)。GPP+PV组患者出现关节损害的概率高于单纯GPP组(χ2=7.55,P<0.05),且伴发高血压的情况较单纯GPP组更为常见(χ2=5.79,P<0.05)。阿维A为最常使用药物。使用免疫抑制剂联合治疗的患者,住院天数最长[(12.57±5.47)d];单独使用生物制剂治疗患者的住院天数[(5.61±2.33)d]最短(P<0.05)。结论性别、年龄及是否合并PV等,均对GPP的临床表型与治疗应答产生重要影响。尽早识别诱发因素、开展精准的亚组干预及生物标志物监测,是改善GPP患者临床结局的关键策略。 展开更多
关键词 泛发性脓疱型银屑病 临床表征 发病诱因 阿维A 生物制剂
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生物制剂治疗妊娠期泛发性脓疱型银屑病1例 认领 引用
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作者 邹雅茹 王君 吕雅琳 《皮肤科学通报》 2026年第1期76-80,共5页
患者女,27岁,孕12周,全身红斑及脓疱反复发作20年,复发加重3月。皮肤科情况:躯干及四肢分布有大小不一的红斑,红斑表面散布针尖至粟粒大小脓疱,局部红斑表面覆盖黄褐色痂皮。诊断:妊娠期泛发性脓疱型银屑病。患者病情在妊娠期间加重,且... 患者女,27岁,孕12周,全身红斑及脓疱反复发作20年,复发加重3月。皮肤科情况:躯干及四肢分布有大小不一的红斑,红斑表面散布针尖至粟粒大小脓疱,局部红斑表面覆盖黄褐色痂皮。诊断:妊娠期泛发性脓疱型银屑病。患者病情在妊娠期间加重,且伴发系统性炎症反应,先后接受依那西普和司库奇尤单抗治疗。患者在依那西普治疗过程中出现药物过敏反应,转用司库奇尤单抗后症状明显缓解,最终成功分娩健康男婴,体现了生物制剂在妊娠期泛发性脓疱型银屑病治疗中的潜在疗效。 展开更多
关键词 泛发性脓疱型银屑病 妊娠 生物制剂 依那西普 司库奇尤单抗
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