É uma forma rara e grave de psoríase, marcada pelo aparecimento de bolinhas de pus (chamadas pústulas) que são estéreis (ou seja, não têm bactérias) e podem surgir de muitas maneiras diferentes no corpo. Todas as principais características da doença (o que ela causa no corpo) se manifestam de forma muito mais intensa. A psoríase pustulosa generalizada é muito diversa na forma como se apresenta: ela pode começar em idades diferentes, ser causada por fatores variados, ter diferentes níveis de gravidade e evoluir de maneiras distintas ao longo da vida. Existem várias formas da doença que se parecem ou se misturam. Existe uma relação entre essas formas e a psoríase em placas (um tipo comum de psoríase), já que algumas pessoas podem apresentar crises de psoríase em placas antes ou depois dos episódios de psoríase pustulosa generalizada. Em outros casos, porém, a psoríase pustulosa generalizada é a única manifestação que a pessoa tem, sem que nunca apareça a psoríase em placas.
Introdução
O que você precisa saber de cara
É uma forma rara e grave de psoríase, marcada pelo aparecimento de bolinhas de pus (chamadas pústulas) que são estéreis (ou seja, não têm bactérias) e podem surgir de muitas maneiras diferentes no corpo. Todas as principais características da doença (o que ela causa no corpo) se manifestam de forma muito mais intensa. A psoríase pustulosa generalizada é muito diversa na forma como se apresenta: ela pode começar em idades diferentes, ser causada por fatores variados, ter diferentes níveis de gravidade e evoluir de maneiras distintas ao longo da vida. Existem várias formas da doença que se parecem ou se misturam. Existe uma relação entre essas formas e a psoríase em placas (um tipo comum de psoríase), já que algumas pessoas podem apresentar crises de psoríase em placas antes ou depois dos episódios de psoríase pustulosa generalizada. Em outros casos, porém, a psoríase pustulosa generalizada é a única manifestação que a pessoa tem, sem que nunca apareça a psoríase em placas.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 19 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 27 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
2 genes identificados com associação a esta condição. Padrão de herança: Autosomal recessive, Not applicable.
Subunit of clathrin-associated adaptor protein complex 1 that plays a role in protein sorting in the late-Golgi/trans-Golgi network (TGN) and/or endosomes. The AP complexes mediate both the recruitment of clathrin to membranes and the recognition of sorting signals within the cytosolic tails of transmembrane cargo molecules. Involved in TLR3 trafficking (PubMed:24791904)
Golgi apparatusCytoplasmic vesicle membraneMembrane, clathrin-coated pit
Psoriasis 15, pustular
A form of pustular psoriasis, a life-threatening disease defined by repeated flares of sudden onset consisting of diffuse erythematous skin eruption characterized by rapid coverage with pustules, high-grade fever, asthenia, marked leukocytosis, and elevated serum levels of C-reactive protein.
Inhibits the activity of interleukin-36 (IL36A,IL36B and IL36G) by binding to receptor IL1RL2 and preventing its association with the coreceptor IL1RAP for signaling. Part of the IL-36 signaling system that is thought to be present in epithelial barriers and to take part in local inflammatory response; similar to the IL-1 system with which it shares the coreceptor. Proposed to play a role in skin inflammation. May be involved in the innate immune response to fungal pathogens, such as Aspergillus
CytoplasmSecreted
Psoriasis 14, pustular
A life-threatening disease defined by repeated flares of sudden onset consisting of diffuse erythematous skin eruption characterized by rapid coverage with pustules, high-grade fever, asthenia, marked leukocytosis, and elevated serum levels of C-reactive protein.
Medicamentos aprovados (FDA)
2 medicamentos encontrados nos registros da FDA americana.
Variantes genéticas (ClinVar)
67 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 188 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
5 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Psoríase pustular generalizada
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
🟢 Recrutando agora
5 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
61 ensaios clínicos encontrados, 12 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 812
[Role and clinical significance of PD-L1+ neutrophils in generalized pustular psoriasis].
Objective To characterize the distribution and functional phenotype of programmed death-ligand 1 (PD-L1) positive neutrophils in generalized pustular psoriasis (GPP) and determine their associations with disease severity and treatment response. Methods We integrated bioinformatic analyses with clinical samples to compare PD-L1 expression on neutrophils from peripheral blood and lesional skin among patients with GPP, healthy controls (HC), and psoriasis vulgaris (PV). PD-L1+ neutrophils were magnetically isolated from HC peripheral blood for in vitro culture, and 24 hours apoptosis was assessed by flow cytometry. The proportion of PD-L1+ neutrophils was quantified by flow cytometry and immunofluorescence and correlated with clinical indices -including GPPASI, high-sensitivity C-reactive protein (hsCRP), neutrophil-to-lymphocyte ratio (NLR), and relapse frequency, as well as clinical response to spesolimab. Results PD-L1 expression on neutrophils was significantly upregulated in both peripheral blood and lesional skin of patients with GPP, showing marked differences compared with HC and PV patients. PD-L1+ neutrophils exhibited delayed apoptosis at 24 hours and a pro-inflammatory phenotype characterized by increased release of IL-36γ-processing enzymes. PD-L1 levels correlated positively with GPPASI, hsCRP, and NLR. The proportion of lesional PD-L1+CD15+ neutrophils was positively associated with relapse frequency. Patients with higher baseline PD-L1 levels demonstrated better clinical responses to spesolimab. Conclusion PD-L1+ neutrophils are enriched in GPP and display delayed apoptosis and inflammatory activation. Their abundance is closely linked to disease activity, systemic inflammation, and biologic response, suggesting a role in shaping GPP immune heterogeneity. PD-L1+ neutrophils are merit consideration as a practical immunologic biomarker for monitoring disease activity and guiding individualized biologic therapy.
Disease Modification of Pustular Psoriasis by Secukinumab: A 20-Year Follow-Up of Von Zumbusch-Type Generalized Pustular Psoriasis With Evolution Into Annular Pustular Psoriasis-A Case Report.
Repeated Episodes of GPP Induced by Respiratory Infections Are Mediated by Loss-of-Function IFIH1.
Generalized pustular psoriasis (GPP) is a severe and recurrent autoinflammatory disease that can be induced by a variety of factors. At present, its etiology and pathogenesis have not yet been fully elucidated. Upper respiratory tract infections are the main predisposing factor in children with GPP, and the MDA5 protein encoded by the IFIH1, functions as a key receptor for sensing upper respiratory tract viruses and may contribute to the induction of antiviral and immunological responses. We performed whole-exome sequencing in 80 pediatric GPP patients and conducted rare variant association analyses against healthy controls. Structural and expression analyses of mutant MDA5 were performed to evaluate mechanistic consequences. Identified IFIH1 variants were functionally characterized using IFN-β luciferase reporter assays, circular dichroism spectroscopy, and protein stability assays. We identified eight IFIH1 rare variants in 10 pediatric GPP patients, accounting for 12.5% of the total cohort. Association analyses revealed a significantly higher prevalence of rare variants of IFIH1 in GPP patients as compared to healthy controls. IFIH1 variation was found to cause structural changes or decreased expression of its encoded protein MDA5, the intrinsic stability of the mutant was lower than that of the wild-type IFIH1. Notably, functional assays demonstrated that these IFIH1 variants (6/8) substantially impaired the production of interferon beta (IFN-β). This study suggests that loss-of-function variants in IFIH1 may be associated with the pathogenesis of recurrent episodes of GPP triggered by URTI. These findings offer a theoretical basis for the development of targeted etiological and pathogenetic preventive measures.
Complications and Laboratory Test Findings Among Patients With Generalized Pustular Psoriasis: A Retrospective Chart Review Study.
Generalized pustular psoriasis (GPP) is a rare, chronic, inflammatory skin disease characterised by widespread eruption of sterile, macroscopic pustules. Patients with GPP can present with multiple comorbidities that may influence treatment. This study aimed to assess the frequency of psoriasis-related complications and non-psoriasis-related comorbidities, and clinical laboratory findings, at the time of GPP diagnosis among patients with GPP. This was a retrospective, longitudinal medical chart review of data from patients with a documented GPP diagnosis attending 29 GPP referral hospitals in Japan. Demographics and clinical characteristics were assessed at baseline (within 6 months prior to and 3 months after GPP diagnosis), including psoriasis-related complications, non-psoriasis-related comorbidities, and clinical laboratory findings. Overall, 205 patients with GPP were included; 48.3% were female, and median age at initial diagnosis was 53 years. Similar proportions of patients had mild (36.1%), moderate (30.7%) and severe (33.2%) GPP at baseline, using Japanese Dermatological Association-GPP severity criteria. Most patients (69.8%) had psoriasis-related complications at baseline, with the most common being psoriasis vulgaris (42.9%) and psoriatic arthritis (26.8%). Non-psoriasis-related comorbidities were present in 69.3% of patients with GPP at baseline, with the most common being hypertension (28.3%), dyslipidaemia (16.6%) and diabetes mellitus (16.1%). There was large variability in laboratory test values between patients. These results demonstrated that, at the time of GPP diagnosis, patients with GPP have multiple burdens of both psoriasis-related complications and non-psoriasis-related comorbidities.
Generalized Pustular Psoriasis: Current Treatment and Innovative Therapies.
Generalized pustular psoriasis (GPP) is a rare, life-threatening auto-inflammatory disease driven by IL-36 pathway dysregulation leading to recurrent flares of widespread erythema, sterile pustules, and systemic symptoms. Real-world data on spesolimab - the first U.S. Food and Drug Administration (FDA)/European Medicines Agency (EMA)-approved GPP therapy - remains scarce, particularly outside Europe. We performed a retrospective case series with prospective follow-up (2010-2025) following Consensus-based Clinical Case Reporting (CARE) guidelines. Nine patients diagnosed according to European Rare and Severe Psoriasis Expert Network (ERASPEN) criteria by board-certified dermatologists were included. Data on demographics, triggers, clinical features, complications, and treatments were collected from electronic medical records. Nine patients (100%) aged 3-61 years, including five (55.6%) female patients, were included. Obesity was the most common comorbidity (n=6, 66.7%). Steroid withdrawal was the most common trigger (n=5, 55.6%). All patients presented with generalized erythema, sterile pustules, and pain; complications observed were acute respiratory distress syndrome (ARDS) in two patients (22.2%) and acute cholangitis in one patient (11.1%). Six flares required inpatient care. Spesolimab achieved 100% flare-free status at 12 months versus 50-75% recurrence with non-IL-36 agents. A tiered treatment algorithm was proposed, prioritizing rapid IL-36 blockade in severe flares. This largest Latin American GPP cohort reveals an earlier onset in pediatric patients and high complication rates. Spesolimab shows superior flare control, but access barriers persist. Standardized protocols and genetic studies in diverse populations are urgently needed.
Publicações recentes
Unusual Presentation of Pustular Psoriasis on the Face: A Case Report.
Juvenile Generalized Pustular Psoriasis: Management in Children-A Case Series.
Janus Kinase Inhibitors for Treatment of Palmoplantar Pustulosis, Generalized Pustular Psoriasis, and Palmoplantar Pustular Psoriasis: A Systematic Review of the Literature.
Successful Treatment of Two Rare Pediatric Keratinization Disorders with Secukinumab: Epidermolytic Ichthyosis and PRP-GPP Overlap.
The IL-36 Cytokine Rheostat: Hierarchical Regulation of Epithelial-Immune Crosstalk and Precision Therapy in Psoriatic and Related Dermatoses.
📚 EuropePMC846 artigos no totalmostrando 198
Novel Heterozygous Hypomorphic MPO Variant Identified in Impetigo Herpetiformis.
The Journal of dermatologyRepeated Episodes of GPP Induced by Respiratory Infections Are Mediated by Loss-of-Function IFIH1.
Immunity, inflammation and diseaseSuccessful Discontinuation of Systemic Corticosteroids With Secukinumab in a Pediatric Case of Generalized Pustular Psoriasis Harboring an IL36RN Mutation.
The Journal of dermatologyComplications and Laboratory Test Findings Among Patients With Generalized Pustular Psoriasis: A Retrospective Chart Review Study.
Experimental dermatologyGeneralized Pustular Psoriasis: Current Treatment and Innovative Therapies.
CureusDe novo Generalized Pustular Psoriasis: A Tofacitinib-Induced Paradoxical Adverse Event.
Indian dermatology online journalReal-World Burden of Generalized Pustular Psoriasis in a French Observational Study: Prevalence, Incidence, Healthcare Resource Utilization, Comorbidities, Treatment Use, and Mortality.
Dermatology and therapyAnnular generalized pustular psoriasis successfully managed with spesolimab induction and secukinumab maintenance.
Medicina clinicaThe Relative Efficacy of Monotherapies for Palmoplantar Pustulosis and Palmoplantar Psoriasis: A Network Meta-Analysis Study of the Palmoplantar Spectrum.
Medicina (Kaunas, Lithuania)Targeting the IL-36 Pathway: Spesolimab as a Therapeutic for Acute Flares of Pustular Psoriasis.
CureusThe IL-36 cytokine family: From barrier immunity to therapeutic target in inflammatory diseases.
Allergology international : official journal of the Japanese Society of AllergologyEconomic Burden of Generalized Pustular Psoriasis: A Population-based Register Study in Finland.
Acta dermato-venereologicaSevere Generalized Pustular Psoriasis With Systemic Capillary Leak Syndrome Successfully Treated With Spesolimab: A Case Report.
The Journal of dermatologyReal-World Evidence of Brodalumab Effectiveness for the Treatment of Psoriasis.
Journal of drugs in dermatology : JDDDupilumab-induced generalized pustular psoriasis in a Chinese woman with atopic dermatitis.
Postepy dermatologii i alergologiiThe efficacy and safety of spesolimab in patients with generalized pustular psoriasis flares: a systematic review and meta-analysis.
Frontiers in medicine[Role and clinical significance of PD-L1+ neutrophils in generalized pustular psoriasis].
Xi bao yu fen zi mian yi xue za zhi = Chinese journal of cellular and molecular immunologyInterleukin-36 receptor antagonist deficiency patient with a novel mutation.
Archives de pediatrie : organe officiel de la Societe francaise de pediatrieGeneralized pustular psoriasis induced by an intraductal pancreatic tumour producing IL-36γ: A case report.
Journal of the European Academy of Dermatology and Venereology : JEADVSustained efficacy of spesolimab following flare-up dose in achieving long-term skin clearance in generalized pustular psoriasis: A case report and review of literature.
JAAD case reportsSevere, Resistant Generalized Pustular Psoriasis-Associated Inflammatory Syndrome Successfully Treated With Adalimumab: A Case Report.
Cureus[Generalized pustulosis after leflunomide].
Dermatologie (Heidelberg, Germany)Treatment of Generalized Pustular Psoriasis with Abrocitinib: A Case Report.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGAcute Myocarditis During a Generalized Pustular Psoriasis Flare: A Possible Pharmacogenetic Interaction in the Setting of Severe Systemic Inflammation?
The Journal of dermatologyReal-World Safety Profile of Spesolimab in Generalized Pustular Psoriasis: Insights From Japan as Part of a Multinational Expanded Access Program (EAP).
The Journal of dermatologyAcute methotrexate-induced cutaneous ulceration in generalized pustular psoriasis: A case report and review of the literature.
JAAD case reportsClinical differentiation between acute generalized exanthematous pustulosis and generalized pustular psoriasis: A multi-center study.
Chinese medical journalSerum neutrophil gelatinase-associated lipocalin as a potential signature for acute generalized exanthematous pustulosis and generalized pustular psoriasis: A case-control study.
JAAD internationalDynamic neutrophil-keratinocyte communication network centered on IL-36/TNFSF15 responses characterizes inflammatory responses in generalized pustular psoriasis.
Nature communicationsClinical phenotype of 504 pustular psoriasis patients from Europe and Egypt: a multi-center observational study.
Clinical and experimental dermatologyDistinct Mortality Risks in Generalized Pustular Psoriasis and Palmoplantar Pustulosis: A Nationwide, Population-Based, Cohort Study.
Dermatology (Basel, Switzerland)Spesolimab Rapidly Resolved Capillary Leak Syndrome Associated with Generalized Pustular Psoriasis.
Acta dermato-venereologicaGeneralized Pustular Psoriasis Confers Increased Risk of Pyoderma Gangrenosum: First Population-Based Evidence.
Dermatology (Basel, Switzerland)Generalized Pustular Psoriasis: Review and Consensus of the Psoriasis Group of the Spanish Academy of Dermatology and Venereology.
Actas dermo-sifiliograficasPatient Experiences on the Diagnosis, Management, and Burden of Generalized Pustular Psoriasis: An International Web Survey and Qualitative Interview Study.
Journal of psoriasis and psoriatic arthritisSuccessful treatment with secukinumab in a 4-month-old infant with severe generalized pustular psoriasis: a case report.
Frontiers in medicineSequential Treatment of Generalized Pustular Psoriasis Combined with Bullous Pemphigoid Using Spesolimab and Dupilumab: A Case Report and Literature Review.
Case reports in dermatologyEpidemiology, clinical characteristics, flares and mortality of generalized pustular psoriasis: a nationwide register study in Finland.
Skin health and diseaseRare coexistence of generalized pustular psoriasis and discoid lupus erythematosus with worsening of lupus lesions after bimekizumab therapy: a case report.
European journal of dermatology : EJDCross-spectrum efficacy of spesolimab in IL-36-mediated dermatoses: a 'case study.
The Journal of dermatological treatmentSafety of secukinumab in pregnant patients with pustular psoriasis: a case report on two successful pregnancies and their offspring outcomes.
The Journal of dermatological treatmentClinical Features and Long-Term Outcomes in 109 Pediatric Generalized Pustular Psoriasis.
Dermatology (Basel, Switzerland)Temporal trends and real-world effectiveness of biologic therapies in generalized pustular psoriasis: a 7-year retrospective cohort study.
The Journal of dermatological treatmentTranscriptomic landscape of generalized pustular psoriasis before and after acitretin/glucocorticoids treatment.
Clinical and experimental medicineBiologic therapy for generalized pustular psoriasis: Real-world comparative effectiveness in a single-center.
Journal of the American Academy of DermatologyAmoxicillin-Triggered Flare of Generalized Pustular Psoriasis in a Patient With Homozygous IL36RN Pathogenic Variant Treated With Spesolimab and Cyclosporin A.
The Journal of dermatologyClinical Profile of Psoriasis Patients With Comorbid Fatty Liver and Risk Factors for Advanced Liver Fibrosis in Those With Metabolic Dysfunction-Associated Steatotic Liver Disease.
The Journal of dermatologySuccessful Treatment of Post-Transplant Generalized Pustular Psoriasis With Apremilast.
The Journal of dermatologyA prospective, observational study on the epidemiology, disease characteristics and treatment outcomes for generalized pustular psoriasis in India.
Clinical and experimental dermatologyCo-Occurrence of RAD21 and TNFAIP3 Mutations in Cornelia de Lange Syndrome with Pustular Psoriasis: Potential Molecular Interactions.
International journal of molecular sciencesHighlights of the Brazilian Society of Dermatology's Brazilian Consensus on Psoriasis.
Anais brasileiros de dermatologiaDual impact of smoking cessation and post-cessation weight change on psoriasis.
Journal of the European Academy of Dermatology and Venereology : JEADVComparison of All-Cause Mortality in Generalized Pustular Psoriasis and Plaque Psoriasis: A Systematic Review and Meta-Analysis.
Dermatology and therapy[Generalized pustular psoriasis: a rare entity in pediatrics].
Boletin medico del Hospital Infantil de MexicoClinical characteristics, treatment outcomes, and relapse prediction in 199 patients with generalized pustular psoriasis: a retrospective cohort study.
The Journal of dermatological treatmentCase Report: Hydroxychloroquine-induced generalized pustular psoriasis in a pediatric patient with systemic lupus erythematosus.
Frontiers in pediatricsUtility and Limitations of Large Language Models to Simplify Online Content on Generalized Pustular Psoriasis.
Rhode Island medical journal (2013)Efficacy and Safety of Tumor Necrosis Factor Alpha Inhibitors for Generalized Pustular Psoriasis of Pregnancy: A Review of Reported Cases.
Journal of cutaneous medicine and surgerySpesolimab for acute-flare generalized pustular psoriasis with severe erythema and plaques: A case report.
MedicineThe roles of serine protease inhibitors in dermatoses.
Frontiers in geneticsBiologic therapies for dermatologic emergencies: A comprehensive review.
Journal of the American Academy of DermatologyGeneralized pustular psoriasis treated with additional biologics after spesolimab: a case series from Japan.
Clinical and experimental dermatologyClinical Features, Treatment Effectiveness and Long-Term Outcomes in Children with Moderate-to-Severe Generalized Pustular Psoriasis: A Retrospective Cohort Study.
Journal of inflammation researchHypocalcemia-related pustulosis: a case report.
Journal of medical case reportsConsiderations for Treating Generalized Pustular Psoriasis (GPP): A Narrative Review.
Dermatology and therapyGeneralized pustular psoriasis in Caucasian patient successfully and rapidly treated with bimekizumab.
Italian journal of dermatology and venereologyEarly Real-Life Experience with Spesolimab in the Treatment of Generalized Pustular Psoriasis: A Case Series of Three Patients Treated in a Compassionate Use Program.
Case reports in dermatologySpesolimab for a child with generalized pustular psoriasis accompanied epilepsy and hyperactivity.
The Journal of dermatological treatmentSuccessful Treatment of Generalized Pustular Psoriasis With Spesolimab in Two Young Children.
Pediatric dermatologyRefractory Pediatric Generalized Pustular Psoriasis Successfully Treated With Spesolimab.
International journal of dermatologyGeneralized pustular psoriasis with purpura and haemorrhagic blisters.
European journal of dermatology : EJDSecukinumab-induced erythema multiforme-like drug eruption in generalized pustular psoriasis: a case report and literature review.
European journal of dermatology : EJDCombination therapy with spesolimab and apremilast for refractory generalized pustular psoriasis: a case report.
Frontiers in medicineSpesolimab for patients with generalized pustular psoriasis flares: Real-world experience in China.
Chinese medical journalGeneralized pustular psoriasis: Advances in pathogenesis, diagnosis, and treatment.
Chinese medical journalHospitalized generalized pustular psoriasis vs. acute generalized exanthematous pustulosis: A comprehensive analysis of clinical manifestations and treatment outcomes.
Chinese medical journalPustular reaction in adult-onset immunodeficiency due to anti-interferon-gamma autoantibodies.
Frontiers in immunologyClinical characteristics, disease burden, and unmet medical needs of generalized pustular psoriasis: Results of the SCRIPTOR study.
Annales de dermatologie et de venereologieEconomic burden and cost drivers of generalized pustular psoriasis: a systematic review.
Health economics reviewReal-world treatment patterns in patients with generalized pustular psoriasis: A US-based claims study.
Journal of the American Academy of DermatologyEthnic Differences of Palmoplantar Pustulosis: A Systematic Review.
American journal of clinical dermatologymicroRNA-223 Expression during Flares in Patients with Generalized Pustular Psoriasis Is Regulated by Spesolimab Treatment.
The Journal of investigative dermatologyHydroxychloroquine-Induced Generalized Pustular Psoriasis Successfully Treated With Secukinumab: A Case Report.
The Journal of dermatologyHealth-related quality of life of adults with generalized pustular psoriasis in Malaysia: a cross-sectional study.
Orphanet journal of rare diseasesPediatric Generalized Pustular Psoriasis Responsive to Colchicine: A Cost-Effective Alternative to Biologics.
Pediatric dermatologyAutoimmune Hemolytic Anemia in a Patient with Generalized Pustular Psoriasis Treated with Brodalumab: A Case Report.
Journal of Nippon Medical School = Nippon Ika Daigaku zasshiSuccessful Treatment of Pediatric Generalized Pustular Psoriasis With Adalimumab: Case Report and Literature Review.
International journal of rheumatic diseasesOutcomes of spesolimab treatment of generalized pustular psoriasis and psoriasis with pustules for 12 weeks: A prospective cohort study from China.
Journal of the American Academy of DermatologyWhole-genome sequencing reveals rare and structural variants contributing to psoriasis and identifies CERCAM as a risk gene.
Cell genomicsSpesolimab Treatment in GPP: Impact of IL-36RN Mutations and Concomitant Plaque Psoriasis.
Clinical, cosmetic and investigational dermatologyGeneralized Pustular Psoriasis as a Systemic Inflammatory Disease: Experience With 38 Japanese Cases Over 15 Years at a Single Institution.
CureusDisease Modification of Pustular Psoriasis by Secukinumab: A 20-Year Follow-Up of Von Zumbusch-Type Generalized Pustular Psoriasis With Evolution Into Annular Pustular Psoriasis-A Case Report.
The Kaohsiung journal of medical sciencesDrug-Induced Generalized Pustular Psoriasis: A Systematic Review.
Journal of cutaneous medicine and surgeryA Global Assessment of Patient Experience and Quality of Life in Generalized Pustular Psoriasis: Results from Interviews and Online Surveys.
Dermatology and therapyClassic Hodgkin lymphoma in a patient treated with ixekizumab for generalized pustular psoriasis.
European journal of dermatology : EJDClinical and genetic characteristics of generalized pustular psoriasis: A retrospective study based on a registry database.
Chinese medical journalDecoding Non-Coding RNA Regulators in DITRA: From Genomic Insights to Potential Biomarkers and Therapeutic Targets.
GenesHealthcare resource utilization and economic burden in generalized pustular psoriasis (GPP) among U.S. adults: A large claims database analysis.
Journal of the American Academy of DermatologyBilateral Multiple Chalazia After Adalimumab Therapy for Uveitis Associated With Generalized Pustular Psoriasis: A Case Report.
CureusAchieving Therapeutic Success with Spesolimab: Two Cases of Drug-Induced Generalized Pustular Psoriasis Initially Mimicking Acute Generalized Exanthematous Pustulosis.
Journal of inflammation researchEpidemiology and Health Care of Generalized Pustular Psoriasis in Germany - Methodology and Outcomes of Claims Data Analysis.
Psoriasis (Auckland, N.Z.)Concurrent superficial epidermolytic ichthyosis and generalized pustular psoriasis - Report of a case, review of the literature, and a proposed pathophysiologic link.
JAAD case reportsTargeting the IL-36 receptor with spesolimab mitigates residual inflammation and prevents generalized pustular psoriasis flares.
The Journal of clinical investigationGeneralized pustular psoriasis in a patient with asthma following dupilumab and tezepelumab therapy.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGDermal adipogenesis protects against neutrophilic skin inflammation during psoriasis pathogenesis.
Cellular & molecular immunologySuccessful Treatment of Adalimumab-Induced Pustular Psoriasis With Guselkumab in a Patient With Hidradenitis Suppurativa.
CureusTargeting IL-23 in Generalized Pustular Psoriasis: Clinical Success With Guselkumab in an Elderly Patient With Comorbidities.
International journal of dermatologyGeneralized Pustular Psoriasis.
The New England journal of medicineNeutrophils in generalized pustular psoriasis: Key players in disease progression.
Chinese medical journalSevere paradoxical generalized pustular psoriasis induced by adalimumab biosimilar successfully treated with brodalumab.
Dermatology reportsUnraveling Psoriasis Flare-up and Dengue Infection.
Journal of psoriasis and psoriatic arthritisAll-Cause Mortality is Higher in Generalized Pustular Psoriasis (GPP) than Plaque Psoriasis and the General Population: A US-Based Claims Analysis.
Journal of psoriasis and psoriatic arthritisTreatment with spesolimab in patients with generalized pustular psoriasis: a review of real-world experience.
Clinical and experimental dermatologySpesolimab in generalized pustular psoriasis complicated by acrodermatitis continua of Hallopeau: a case report and mechanistic insights.
Frontiers in immunologyParadoxical development of prurigo nodularis during ixekizumab therapy in a patient with generalized pustular psoriasis and psoriatic arthritis.
European journal of dermatology : EJDGeneralized Pustular Psoriasis During Early Pregnancy Successfully Treated with Spesolimab: A Case Report.
International medical case reports journalTreatment Status for Generalized Pustular Psoriasis in Japanese Patients: A Retrospective Chart Review.
Dermatology and therapyGeneralized Pustular Psoriasis with Cushing's Syndrome: A Case of Effective Spesolimab Treatment.
Biologics : targets & therapyAnnular pustular psoriasis: A heterogeneous subtype of generalized pustular psoriasis.
Chinese medical journalUpdate on Generalized Pustular Psoriasis.
Acta medica portuguesaGeneralized Pustular Psoriasis-Like Eruption Triggered by a G-CSF-Producing Lung Adenocarcinoma.
The Journal of dermatologyGeneralized pustular psoriasis (von Zumbusch) flares successfully treated with Spesolimab. Report of two cases and review of the literature.
Anais brasileiros de dermatologiaImmunoproteomic Response to IL-17A Blockade in a Patient With Generalized Pustular Psoriasis: New Insights From Plasma Olink Profiling.
The Journal of dermatologyMedications Associated With Generalized Pustular Psoriasis Flares: A Retrospective Pharmacovigilance Study.
Journal of cutaneous medicine and surgeryEpidemiology and admission outcomes of generalized pustular psoriasis: a report from the national inpatient sample.
Archives of dermatological researchSuccessful treatment of generalized pustular psoriasis with chronic hepatitis B using spesolimab: A case report.
MedicineEffective Spesolimab Treatment for Generalized Pustular Psoriasis Masquerading as Acute Generalized Exanthematous Pustulosis: A Case Report.
CureusA Case of Parkinsonism Manifested by Exacerbation of Generalized Pustular Psoriasis.
The Journal of dermatologySpesolimab for the Treatment of Generalized Pustular Psoriasis Flares: An Observational, Retrospective, and Multicenter Study of Effectiveness and Safety in Real-World Clinical Practice.
International journal of dermatologyPediatric Patient With Generalized Pustular Psoriasis With MEFV Gene Variant: Successful Treatment With Adalimumab.
Pediatric dermatologyReal-World Evidence on the Effectiveness and Safety of Spesolimab in the Treatment of Generalized Pustular Psoriasis Flares: A Case Series.
BioDrugs : clinical immunotherapeutics, biopharmaceuticals and gene therapyJourney of Patients with Psoriasis in an Italian Tertiary Centre, an 11-year Retrospective Analysis.
Acta dermato-venereologicaSuccessful Management of Refractory Generalized Pustular Psoriasis With Spesolimab.
American journal of therapeuticsSafety and effectiveness of secukinumab in Japanese patients with generalized pustular psoriasis: A post-marketing surveillance.
The Journal of dermatologyEpidemiological, Genetic, Clinical, and Treatment Differences of Generalized Pustular Psoriasis and Acrodermatitis Continua of Hallopeau Across Ethnicities: A Systematic Review.
American journal of clinical dermatologyGeneralized Pustular Psoriasis Brazilian Experts Survey: Challenges in Disease Management.
Dermatology and therapyAcrodermatitis Continua of Hallopeau and Generalised Pustular Psoriasis: Case Reports of Two Different Manifestations of IL36RN Mutation in Siblings.
Psoriasis (Auckland, N.Z.)Efficacy and safety of spesolimab in the treatment of generalized pustular psoriasis in elderly patients.
Annales de dermatologie et de venereologieGeneralized Pustular Psoriasis with Breast Cancer Successfully Treated with Spesolimab Causing Neutrophilia: A Case Report.
Acta dermato-venereologicaAbrocitinib Treatment for Localized Type of Generalized Pustular Psoriasis: A Case Report.
Clinical, cosmetic and investigational dermatologyTreating Generalized Pustular Psoriasis (GPP): Timing and Rationale for Biologic Treatment Switching-A Japanese e-Delphi Survey.
Dermatology and therapyReal-Life Analysis of Therapeutic Management and Its Correlation with the Dermatology Life Quality Index Score in 108 Patients with Pustular Psoriasis: An Italian Monocenter Study.
Dermatology practical & conceptualSuccessful treatment of generalized pustular psoriasis during pregnancy with secukinumab in a patient hypersensitive to spesolimab: a case report.
The Journal of dermatological treatmentJust the facts: diagnosis and management of generalized pustular psoriasis.
CJEMProteomic Profiling and Clinical Insights: The Role of MMP9 in Differentiating Psoriasis Vulgaris from Generalized Pustular Psoriasis.
Journal of inflammation researchCurrent status of generalized pustular psoriasis: Findings from a multicenter hospital-based survey of 127 Chinese patients.
Chinese medical journalSafety and effectiveness of ixekizumab in Japanese patients with psoriasis vulgaris, psoriatic arthritis, generalized pustular psoriasis, and erythrodermic psoriasis: Post-marketing surveillance.
The Journal of dermatologyFrom the Masterclasses in Dermatology 2024 Meeting: Updates in Psoriasis Treatments.
The Journal of clinical and aesthetic dermatologyExploring the Significance of Eosinophil Infiltration in Diagnosis of Psoriasis: A Cross-sectional Analysis.
Iranian journal of pathologyEffect of spesolimab on sustained disease control in patients with generalized pustular psoriasis: Post hoc analysis of the EFFISAYIL 2 study.
Journal of the American Academy of DermatologySpesolimab for the Treatment of Generalized Pustular Psoriasis Flares.
Journal of drugs in dermatology : JDDCase report: Efficacy of tofacitinib in the treatment of generalized pustular psoriasis.
Frontiers in medicineEpidemiology of generalized pustular psoriasis in Germany: Analyzing factors influencing prevalence estimates health insurance data.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGQuality of life of patients with pustular psoriasis is inferior to that of patients with plaque psoriasis in Japan: A multicenter study with questionnaires, the short Form-36, and other patient-reported outcomes.
The Journal of dermatologyCharacteristics and Outcomes of Generalized Pustular Psoriasis Patients Managed Through Inpatient Asynchronous Teledermatology: A Retrospective Study.
Telemedicine reportsIL-36-driven pustulosis: Transcriptomic signatures match between generalized pustular psoriasis (GPP) and acute generalized exanthematous pustulosis (AGEP).
The Journal of allergy and clinical immunologyEfficacy of granulocyte and monocyte adsorptive apheresis on skin and joint manifestations of palmoplantar pustulosis with pustulotic arthro-osteitis: A multicentric, prospective, observational study.
The Journal of dermatologyIxekizumab as a successful treatment in pediatric generalized pustular psoriasis.
Italian journal of pediatricsReal-world clinical characteristics and treatment patterns of generalized pustular psoriasis flares.
Journal of the American Academy of DermatologyLiterature review and expert opinion on diagnosis and current management of generalized pustular psoriasis.
Expert opinion on biological therapyGeneralized pustular psoriasis: a multicentric study on patient characteristics and clinical burden.
Orphanet journal of rare diseasesAnalysis of the German Compassionate Use Program on spesolimab in patients with generalized pustular psoriasis: evidence outside of clinical trials.
European journal of dermatology : EJDEffectiveness and Safety of Spesolimab in Patients with Generalized Pustular Psoriasis: A Single-centre Retrospective Study.
Acta dermato-venereologicaCrusted Scabies in a Patient being Treated with Spesolimab for Generalized Pustular Psoriasis.
Acta dermato-venereologicaManagement of Chronic Generalized Pustular Psoriasis: A Review and Expert Opinion.
Journal of psoriasis and psoriatic arthritisEfficacy and safety of risankizumab for the treatment of patients with plaque type psoriasis.
Italian journal of dermatology and venereologyHealthcare Resource Utilization Among Patients With Generalized Pustular Psoriasis: The Impact of Flares and Disease Severity.
Journal of psoriasis and psoriatic arthritisGlobal Delphi consensus on treatment goals for generalized pustular psoriasis.
The British journal of dermatologyComments on Consensus for Generalized Pustular Psoriasis-Reply.
JAMA dermatologyComments on Consensus for Generalized Pustular Psoriasis.
JAMA dermatologySuccessful Treatment of Pediatric Generalized Pustular Psoriasis with Secukinumab.
Clinical, cosmetic and investigational dermatologyAcute Generalized Exanthematous Pustulosis Induced by Hyaluronic Acid Knee Injection: A Case Report.
Journal of emergency nursingEruption of Generalized Pustular Psoriasis Following Initiation of Adalimumab for Hidradenitis Suppurativa: A Case Report.
CureusLong-Term efficacy and safety analysis of secukinumab in the treatment of pediatric generalized pustular psoriasis: a real-world study.
The Journal of dermatological treatmentRecibokibart, an anti-IL-36 receptor monoclonal antibody, for treating generalized pustular psoriasis: Phase 1b trial.
Journal of the European Academy of Dermatology and Venereology : JEADVRapid improvement in a recurrent generalized pustular psoriasis patient ineffective to Ixekizumab following Spesolimab therapy.
Anais brasileiros de dermatologiaGeneralized pustular psoriasis: immunological mechanisms, genetics, and emerging therapeutics.
Trends in immunologyEtanercept: A viable treatment option for young children with generalized pustular psoriasis.
Pediatric investigationEfficacy of secukinumab in Takayasu arteritis with myocardial infarction complicated with generalized pustular psoriasis: A case report.
MedicineUpdated genetic background of generalized pustular psoriasis as an autoinflammatory keratinization disease.
The Journal of dermatologyEffectiveness and safety of spesolimab in treating generalized pustular psoriasis: A comparative analysis of randomized controlled trials.
Indian journal of pharmacologyUnveiling the skin microbial guardians and assailants in psoriasis subtypes: a Mendelian randomization study.
Archives of dermatological researchReal-World Experience of Spesolimab for Generalized Pustular Psoriasis Flares in Adult Patients: A Multicenter Retrospective Study.
American journal of clinical dermatologyCharacteristics of Patients with Generalized Pustular Psoriasis: A Report of the Polish Generalized Pustular Psoriasis Group.
Dermatology and therapyInnovations in Psoriasis.
Dermatologic clinicsExploring the Chronic Nature of Generalized Pustular Psoriasis [Podcast].
Clinical, cosmetic and investigational dermatologySuccessful Treatment of Pediatric Generalized Pustular Psoriasis (GPP) with Spesolimab: 5 Case Reports and Evaluations of Circulating IL-36 Levels.
Journal of inflammation researchBiologics for generalized pustular psoriasis: a systematic review and single-arm meta-analysis.
Frontiers in immunologyLong-term remission and normal pregnancy with successful delivery in a patient with generalized pustular psoriasis after spesolimab.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGAsia-Pacific consensus recommendations on the management of generalized pustular psoriasis.
The Journal of dermatologyIL36RN Mutations and Correlated Characteristics in Generalized Pustular Psoriasis Patients in Can Tho City, Vietnam: A Cross-Sectional Study.
Dermatology (Basel, Switzerland)Spesolimab, the first-in-class anti-IL-36R antibody: From bench to clinic.
The Journal of dermatologyComparative Efficacy and Safety of Ustekinumab and Secukinumab in the Treatment of Generalized Pustular Psoriasis: A 48-Week Retrospective Cohort Study with Genetic Background Analysis.
Journal of inflammation researchGeneralized pustular psoriasis patient with a heterozygous hypomorphic MPO variant refractory to intravenous spesolimab.
The Journal of dermatologyCorrigendum to Acute Respiratory Distress Syndrome in a Carrier of an Interleukin-36 Receptor Antagonist Mutation With Generalized Pustular Psoriasis.
Journal of psoriasis and psoriatic arthritis[Translated article] Elderly-Onset Generalized Pustular Psoriasis in the Elderly: A 5-Case Series.
Actas dermo-sifiliograficasConsiderations for defining and diagnosing generalized pustular psoriasis.
Journal of the European Academy of Dermatology and Venereology : JEADVPlaque psoriasis, generalized pustular psoriasis, palmoplantar pustulosis, and acrodermatitis continua of Hallopeau successfully treated with bimekizumab: a promising therapeutic approach.
European journal of dermatology : EJDAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- [Role and clinical significance of PD-L1+ neutrophils in generalized pustular psoriasis].Xi bao yu fen zi mian yi xue za zhi = Chinese journal of cellular and molecular immunology· 2026· PMID 41620967mais citado
- Disease Modification of Pustular Psoriasis by Secukinumab: A 20-Year Follow-Up of Von Zumbusch-Type Generalized Pustular Psoriasis With Evolution Into Annular Pustular Psoriasis-A Case Report.
- Repeated Episodes of GPP Induced by Respiratory Infections Are Mediated by Loss-of-Function IFIH1.
- Complications and Laboratory Test Findings Among Patients With Generalized Pustular Psoriasis: A Retrospective Chart Review Study.
- Generalized Pustular Psoriasis: Current Treatment and Innovative Therapies.
- Unusual Presentation of Pustular Psoriasis on the Face: A Case Report.
- Juvenile Generalized Pustular Psoriasis: Management in Children-A Case Series.
- Janus Kinase Inhibitors for Treatment of Palmoplantar Pustulosis, Generalized Pustular Psoriasis, and Palmoplantar Pustular Psoriasis: A Systematic Review of the Literature.
- Successful Treatment of Two Rare Pediatric Keratinization Disorders with Secukinumab: Epidermolytic Ichthyosis and PRP-GPP Overlap.
- The IL-36 Cytokine Rheostat: Hierarchical Regulation of Epithelial-Immune Crosstalk and Precision Therapy in Psoriatic and Related Dermatoses.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:247353(Orphanet)
- MONDO:0100491(MONDO)
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q5532501(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
