2023
DOI: 10.2147/ccid.s407812
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Use of Biological Therapies for the Management of Pustular Psoriasis: A New Era?

Abstract: Generalized pustular psoriasis (GPP) is a severe and rare form of psoriasis, being a potentially life-threatening condition, characterized by recurring episodes or flares of widespread cutaneous erythema with macroscopic sterile pustules. An irregular innate immune response is linked to GPP, which is considered an auto-inflammatory disorder, while innate and adaptive immunopathogenic responses are involved in psoriasis pathogenesis. In consequence, different cytokine cascades have been suggested to be mainly i… Show more

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Cited by 19 publications
(9 citation statements)
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References 147 publications
(265 reference statements)
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“…Current treatment of GPP flares often relies on the off-label use of medications licensed for moderate to severe plaque psoriasis, but the limited evidence that supports this practice is derived from case reports and small single-arm studies [ 9 , 14 , 20 ]. Actually, while it can co-exist with plaque psoriasis and is traditionally classified as a subtype of psoriasis, GPP stands apart due to its distinctive clinical and immune-histopathologic features, as well as genetics [ 1 , 11 ].…”
Section: Discussionmentioning
confidence: 99%
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“…Current treatment of GPP flares often relies on the off-label use of medications licensed for moderate to severe plaque psoriasis, but the limited evidence that supports this practice is derived from case reports and small single-arm studies [ 9 , 14 , 20 ]. Actually, while it can co-exist with plaque psoriasis and is traditionally classified as a subtype of psoriasis, GPP stands apart due to its distinctive clinical and immune-histopathologic features, as well as genetics [ 1 , 11 ].…”
Section: Discussionmentioning
confidence: 99%
“…Currently, established international guidelines for the pharmacologic treatment of GPP are lacking, mainly due to the scarcity of high-quality evidence data on its efficacy. The management of GPP has been globally based on recommendations designed for plaque psoriasis [ 14 , 19 , 20 ]. In fact, before spesolimab approval in 2022, no specific treatments for GPP were approved by the FDA or EMA.…”
Section: Overview Of Current Treatment Options In Generalized Pustula...mentioning
confidence: 99%
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“… 1 , 3 Other phenotypes include guttate, erythrodermic, pustular, inverse, and palmoplantar psoriasis. 1 , 3 , 4 …”
Section: Introductionmentioning
confidence: 99%
“…1 The release of cytokines such as IL-17 and TNF-alpha cause the activation of neutrophils, Th17 cells, and dendritic cells, leading to neutrophilic infiltration. 4 Additionally, the IL-36 inhibitor spesolimab, which recently received FDA approval for GPP management, could be considered. The IL-36 axis drives the release of these cytokines and plays a central role in stimulating pustular formation.…”
mentioning
confidence: 99%