Treatments and outcomes of generalized pustular psoriasis: A cohort of 1516 patients in a nationwide inpatient database in Japan

Miyachi, Hideaki; Konishi, Takaaki; Kumazawa, Ryosuke; Matsui, Hiroki; Shimizu, Sayuri; Fushimi, Kiyohide; Matsue, Hiroyuki; Yasunaga, Hideo

doi:10.1016/j.jaad.2021.06.008

Cited by 71 publications

(111 citation statements)

References 29 publications

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“…Augey et al [ 10 ] questioned dermatologists and reported on 99 patients with GPP with a male:female ratio was 0.77; the mean age of incident cases was 59.7% and a mortality rate of 2% was indicated. Miyachi et al [ 38 ] reported on 1516 patients with GPP, of whom 853 were male and 663 were female; a mortality rate of 4.2% was indicated) Blank cells indicate when data are not presented (or not presented in a calculable format) in the cited study. Individual overall study populations may include different forms of GPP, including juvenile GPP or other pustular disease GPP generalized pustular psoriasis a Includes five patients with palmoplantar pustulosis and four patients with acrodermatitis continua of Hallopeau b Study of pediatric patients with GPP/patients with juvenile onset c Three-quarters of patients were aged over 40 years at diagnosis d Mean age at admittance to the hospital ward e Median age of diagnosis f Data for patients with acute GPP only g Baker and Ryan did not provide a mean pre-pustular duration, but for patients with preceding psoriasis “many patients [passed] 10 or 20 or more years before GPP developed” [ 4 ] h Among patients with psoriasis preceding acute GPP (24/35 patients) i 28/31 patients with GPP presented with systemic inflammation j Mortality data presented by Ryan and Baker, using the same patient cohort [ 40 ]…”

Section: Clinical Characteristics and Course Of Gppmentioning

confidence: 99%

Section: Clinical Characteristics and Course Of Gppmentioning

confidence: 99%

“…Generalized pustular psoriasis flares can be severe and potentially life threatening. An estimated 50% of GPP flares require hospitalization [ 38 ] and a patient with GPP will require hospitalization for a flare at least once every 5 years [ 3 ]. The duration of inpatient stay for a GPP flare is reported to be 10–14 days [ 39 ].…”

Section: Clinical Characteristics and Course Of Gppmentioning

confidence: 99%

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Clinical Course and Characteristics of Generalized Pustular Psoriasis

2022

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Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening disease characterized by episodes of widespread sterile macroscopic pustules, with or without systemic inflammation and/or plaque psoriasis. Multiple GPP subtypes have been described, from acute GPP of von Zumbusch to milder, annular pustular psoriasis. Generalized pustular psoriasis mainly affects adults, with a female preponderance, but juvenile GPP also occurs. Flares are a hallmark of GPP and may occur de novo or be provoked by triggers, including withdrawal of systemic corticosteroids, infections, stress, pregnancy, and menstruation. Severity of flares varies widely between patients, and between flares in an individual patient. Significant flares are often accompanied by systemic symptoms, notably fever, general malaise, and extracutaneous manifestations such as arthritis, uveitis, and neutrophilic cholangitis. Common laboratory abnormalities include neutrophilia, elevated C-reactive protein levels, hypocalcemia, and abnormal liver function tests. The clinical course of GPP is highly variable; it can be a relapsing disease with recurrent flares and no pustulation between flares or a persistent disease with perpetual mild pustulation punctuated with flares of greater severity. Patients may have multiple flares per year or a flare every few years. Most flares last 2–5 weeks and approximately 50% require hospitalization. Life-threatening complications include sepsis and renal, hepatic, respiratory, and heart failure. Reported mortality rates are 2–16%.

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Section: Clinical Characteristics and Course Of Gppmentioning

confidence: 99%

Section: Clinical Characteristics and Course Of Gppmentioning

confidence: 99%

Section: Clinical Characteristics and Course Of Gppmentioning

confidence: 99%

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Clinical Course and Characteristics of Generalized Pustular Psoriasis

2022

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“…Dapsone has also been proposed as a possible therapeutic option [30,154]. Systemic corticosteroids are usually discouraged-with a few exceptions-due to the increased risk of pustulation, as well as flares during treatment or upon discontinuation [30,155], although a recent study has shown low rates of psoriasis flare in such circumstances [156].…”

Section: Treatment Of Generalized Pustular Psoriasismentioning

confidence: 99%

“…IL-17 inhibitor use was associated with comparable in-hospital mortality and morbidity to those of TNF inhibitors. Indeed, about half of the patients in the biologics group were treated with concomitant oral agents, sometimes in addition to systemic corticosteroids [ 155 ].…”

Section: Treatmentmentioning

confidence: 99%

Pustular Psoriasis: From Pathophysiology to Treatment

Genovese

Moltrasio

Cassano³

et al. 2021

Biomedicines

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Pustular psoriasis (PP) is a clinicopathological entity encompassing different variants, i.e., acute generalized PP (GPP), PP of pregnancy (impetigo herpetiformis), annular (and circinate) PP, infantile/juvenile PP, palmoplantar PP/palmoplantar pustulosis, and acrodermatitis continua of Hallopeau (ACH), which have in common an eruption of superficial sterile pustules on an erythematous base. Unlike psoriasis vulgaris, in which a key role is played by the adaptive immune system and interleukin (IL)-17/IL-23 axis, PP seems to be characterized by an intense inflammatory response resulting from innate immunity hyperactivation, with prominent involvement of the IL-36 axis. Some nosological aspects of PP are still controversial and debated. Moreover, owing to the rarity and heterogeneity of PP forms, data on prognosis and therapeutic management are limited. Recent progresses in the identification of genetic mutations and immunological mechanisms have promoted a better understanding of PP pathogenesis and might have important consequences on diagnostic refinement and treatment. In this narrative review, current findings in the pathogenesis, classification, clinical features, and therapeutic management of PP are briefly discussed.

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Understanding Flares in Patients With Generalized Pustular Psoriasis Documented in US Electronic Health Records

et al. 2022

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Key Points Question What are the differences in treatment between patients with generalized pustular psoriasis (GPP) with flares and those without flares? Findings In this cohort study comprising 1535 patients with GPP, with 271 of them having 513 documented flares, patients with flares had a higher comorbidity burden, were almost 3 times more likely to have inpatient visits, were more than twice as likely to have emergency department visits, and had higher use of almost all treatment classes. Despite this high level of severity, advanced treatments for GPP were very rarely used during flare episodes. Meaning This study suggests that there is a significant unmet need in the current treatment of GPP and its flares.

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Treatments and outcomes of generalized pustular psoriasis: A cohort of 1516 patients in a nationwide inpatient database in Japan

Cited by 71 publications

References 29 publications

Clinical Course and Characteristics of Generalized Pustular Psoriasis

Clinical Course and Characteristics of Generalized Pustular Psoriasis

Pustular Psoriasis: From Pathophysiology to Treatment

Understanding Flares in Patients With Generalized Pustular Psoriasis Documented in US Electronic Health Records

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