Generalized pustular psoriasis is a disease distinct from psoriasis vulgaris: evidence and expert opinion

Bachelez, H.; Barker, Jonathan; Burden, A. David; Navarini, Alexander A.; Krueger, James G.

doi:10.1080/1744666x.2022.2116003

Cited by 34 publications

(35 citation statements)

References 126 publications

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“…The condition currently known as GPP was first described by Leopold von Zumbusch in 1910. Since its first description, clinical characteristics have not been consistently defined and several descriptions and different diagnostic criteria have been used [4][5][6][7][8][9][10][11][12][13][14].…”

Section: Clinical Characteristics and Diagnosismentioning

confidence: 99%

“…In addition, clinical awareness is limited and the difficulty reaching a diagnosis challenges the estimation of disease prevalence. A high variability in prevalence between different ethnicities and geographical regions has been reported [9][10][11][12][13][14][15].…”

Section: Epidemiologymentioning

confidence: 99%

“…GPP can present at any age, from childhood to old age, but the median reported age at diagnosis is around 50 years [9][10][11][12][13][14][15]. In general, female preponderance has been observed in GPP patients.…”

Section: Epidemiologymentioning

confidence: 99%

“…Globally, prevalence of GPP can be estimated as 1-7 cases per million persons [8][9][10][11][12][13][14][15]. However, data are variable, and estimations range from 1.76 cases per million in France to 7.46 cases per million in Japan, 180 cases per million [15].…”

Section: Epidemiologymentioning

confidence: 99%

“…Extracutaneous manifestations, including arthritis, uveitis, and neutrophilic cholangitis, can also appear. Laboratory abnormalities, including neutrophilia, elevated CRP levels, abnormal liver function tests, hypocalcemia, and hypoalbuminemia, may be associated with flares [4][5][6][9][10][11][12][13].…”

Section: Clinical Coursementioning

confidence: 99%

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Generalized Pustular Psoriasis: A Review on Clinical Characteristics, Diagnosis, and Treatment

Díaz

Daudén

Carrascosa

et al. 2023

Dermatol Ther (Heidelb)

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Generalized pustular psoriasis (GPP) is a rare, chronic, and severe inflammatory skin disorder characterized by sudden eruption of sterile pustules, often accompanied by systemic inflammation. GPP flares can be life-threatening if untreated, owing to potential serious complications such as sepsis and cardiovascular failure. Diagnosis and clinical measurement of disease severity in GPP are often difficult. Lack of standardized criteria in the international guidelines and the heterogeneity of cutaneous and extracutaneous symptoms make the diagnosis of GPP difficult. Clinical criteria for description and diagnosis of pustular conditions, including GPP, are variable and there is no specific agreement on commonly sustained concepts. Differentiation of GPP from other similar conditions/diseases is important and requires careful assessments. The evidence that supports current topical or systemic therapies is largely based on case reports and small studies. Some biologic agents that target key cytokines involved in the activation of inflammatory pathways have been used as treatments for GPP. Recently, spesolimab, an IL-36R antagonist, has been approved in the USA and Japan for the treatment of GPP flares in adults, but there are no currently approved treatments for GPP in Europe. The IL-36 pathway has recently emerged as a central axis driving the pathogenic inflammatory mechanisms of GPP. Biologic agents that inhibit the IL-36 pathway have shown efficacy and safety in patients with GPP, addressing a generally considered unmet medical need.

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Section: Clinical Characteristics and Diagnosismentioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

Section: Clinical Coursementioning

confidence: 99%

See 3 more Smart Citations

Generalized Pustular Psoriasis: A Review on Clinical Characteristics, Diagnosis, and Treatment

Díaz

Daudén

Carrascosa

et al. 2023

Dermatol Ther (Heidelb)

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Clinical and Disease Burden of Patients with Generalized Pustular Psoriasis: A Review of Real-World Evidence

Bhutani,

Farberg

2024

Dermatol Ther (Heidelb)

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Generalized pustular psoriasis (GPP) is a chronic, rare, and potentially life-threatening disease. There is limited understanding of patient characteristics in GPP and their correlation with disease progression or healthcare resource utilization. Our review aims to examine real-world evidence on these characteristics and the associated disease burden as related to economic and quality of life factors. Results showed that most patients with GPP experienced flares once a year, lasting from 2 weeks to 3 months, with > 80% of patients having residual disease post-flare, with/without treatment, indicating the long-term nature of GPP. The impact of GPP on patients’ daily activities was significant, even in the absence of a flare. GPP adversely affected mental health, and anxiety and depression were reported regularly. Patients with GPP had more comorbidities, were prescribed more medication, and had more inpatient and outpatient visits than in matched plaque psoriasis or general population cohorts. Improving the education of healthcare providers in diagnosing GPP, defining disease flares, and managing the disease, as well as making globally accepted clinical guidelines for GPP treatment available, could help to reduce the burden on patients with GPP. Effective therapies that control and prevent GPP flares and manage chronic disease are needed.

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Current Treatments for Generalized Pustular Psoriasis: A Narrative Summary of a Systematic Literature Search

Puig,

Fujita,

Thaçi

et al. 2024

Dermatol Ther (Heidelb)

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Generalized pustular psoriasis is a disease distinct from psoriasis vulgaris: evidence and expert opinion

Cited by 34 publications

References 126 publications

Generalized Pustular Psoriasis: A Review on Clinical Characteristics, Diagnosis, and Treatment

Generalized Pustular Psoriasis: A Review on Clinical Characteristics, Diagnosis, and Treatment

Clinical and Disease Burden of Patients with Generalized Pustular Psoriasis: A Review of Real-World Evidence

Current Treatments for Generalized Pustular Psoriasis: A Narrative Summary of a Systematic Literature Search

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