The update of treatment strategies in pediatrics with generalized pustular psoriasis in China

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.

Understanding the Pathogenesis of Generalized Pustular Psoriasis Based on Molecular Genetics and Immunopathology

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.

Characterization of Generalized Pustular Psoriasis in Northwest China: A Single-Center Retrospective Study

Objective:This study was performed to investigate the clinical characteristics of patients with generalized pustular psoriasis(GPP)in Northwest China.Methods:The clinical data of patients with GPP were retrospectively collected in the Second Affiliated Hospital of Xi’an Jiaotong University from January 1,2017,to December 31,2021 and analyzed using the chi-square test and Mann-Whitney U test.Results:In total,179 hospitalized patients were included.The male:female ratio was 1.16:1.00,and the mean age at onset was 35.05±19.11 years.Psoriasis vulgaris was also present in 76.0%of patients,and a family history of psoriasis was present in 15.6%.The mean duration of hospitalization was 10.67±4.31 days,and the mean duration of flares was 29.58±24.32 days.Infections and suspected drugs were predisposing factors.A fever developed in 54.7%of patients,and pruritus developed in 70.9%.Some patients had involvement of the nails(38.0%),scalp(46.9%),and tongue(4.5%).Cardiovascular disease,hypertension,and gallbladder-related disease were common comorbidities.The efficacy of acitretin was 84.7%,that of methotrexate was 66.7%,and that of cyclosporine was 100%.Fifteen patients were treated with secukinumab or adalimumab and responded well.The mean response time was 6.34±2.91 days based on the combination treatments.The mean duration of the treatment regimen was 111.35±94.25 days,and approximately 46.6%(n=131)of patients developed recurrence.Conclusion:Our retrospective study showed that most cases of GPP were accompanied by psoriasis vulgaris and associated with fever or pruritus.Acitretin had good therapeutic efficacy,but recurrence should be noted.Biologics are increasingly becoming effective treatments,but their superiority and safety need further research.

Childhood pustular psoriasis--A case of a patient treated with traditional Chinese medicine

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.