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Unmet Educational Needs and Clinical Practice Gaps in the Management of Generalized Pustular Psoriasis: Global Perspectives from the Front Line

INTRODUCTION: Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening, neutrophilic, autoinflammatory skin disease characterised by recurrent flares of generalised sterile pustules and associated systemic features. Inconsistent diagnostic criteria and a lack of approved therapie...

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Autores principales: Strober, Bruce, Leman, Joyce, Mockenhaupt, Maja, Nakano de Melo, Juliana, Nassar, Ahmed, Prajapati, Vimal H., Romanelli, Paolo, Seneschal, Julien, Tsianakas, Athanasios, Wei, Lee Yoong, Yasuda, Masahito, Yu, Ning, Hernandez Daly, Ana C., Okubo, Yukari
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Healthcare 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8850517/
https://www.ncbi.nlm.nih.gov/pubmed/34904208
http://dx.doi.org/10.1007/s13555-021-00661-2
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author Strober, Bruce
Leman, Joyce
Mockenhaupt, Maja
Nakano de Melo, Juliana
Nassar, Ahmed
Prajapati, Vimal H.
Romanelli, Paolo
Seneschal, Julien
Tsianakas, Athanasios
Wei, Lee Yoong
Yasuda, Masahito
Yu, Ning
Hernandez Daly, Ana C.
Okubo, Yukari
author_facet Strober, Bruce
Leman, Joyce
Mockenhaupt, Maja
Nakano de Melo, Juliana
Nassar, Ahmed
Prajapati, Vimal H.
Romanelli, Paolo
Seneschal, Julien
Tsianakas, Athanasios
Wei, Lee Yoong
Yasuda, Masahito
Yu, Ning
Hernandez Daly, Ana C.
Okubo, Yukari
author_sort Strober, Bruce
collection PubMed
description INTRODUCTION: Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening, neutrophilic, autoinflammatory skin disease characterised by recurrent flares of generalised sterile pustules and associated systemic features. Inconsistent diagnostic criteria and a lack of approved therapies pose serious challenges to GPP management. Our objectives were to discuss the challenges encountered in the care of patients with GPP and identify healthcare provider (HCP) educational needs and clinical practice gaps in GPP management. METHODS: On 24 July 2020, 13 dermatologists from 10 countries (Brazil, Canada, China, Egypt, France, Germany, Japan, Malaysia, the UK and the USA) attended a workshop to share experiences in managing patients with GPP. Educational needs and clinical practice gaps grouped according to healthcare system level were discussed and ranked using interactive polling. RESULTS: Lack of experience of GPP among HCPs was identified as an important individual HCP-level clinical practice gap. Limited understanding of the presentation and pathogenesis of GPP among non-specialists means misdiagnosis is common, delaying referral and treatment. In countries where patients may present to general practitioners or emergency department HCPs, GPP is often mistaken for an infection. Among dermatologists who can accurately diagnose GPP, limited knowledge of treatments may necessitate referral to a colleague with more experience in GPP. At the organisational level, important needs identified were educating emergency department HCPs to recognise GPP as an autoinflammatory disease and improving communication, cooperation and definitions of roles within multidisciplinary teams supporting patients with GPP. At the regulatory level, robust clinical trial data, clear and consistent treatment guidelines and approved therapies were identified as high priorities. CONCLUSIONS: The educational imperative most consistently identified across the participating countries is for HCPs to understand that GPP can be life-threatening if appropriate treatment initiation is delayed, and to recognise when to refer patients to a colleague with more experience of GPP management. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s13555-021-00661-2.
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spelling pubmed-88505172022-02-23 Unmet Educational Needs and Clinical Practice Gaps in the Management of Generalized Pustular Psoriasis: Global Perspectives from the Front Line Strober, Bruce Leman, Joyce Mockenhaupt, Maja Nakano de Melo, Juliana Nassar, Ahmed Prajapati, Vimal H. Romanelli, Paolo Seneschal, Julien Tsianakas, Athanasios Wei, Lee Yoong Yasuda, Masahito Yu, Ning Hernandez Daly, Ana C. Okubo, Yukari Dermatol Ther (Heidelb) Original Research INTRODUCTION: Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening, neutrophilic, autoinflammatory skin disease characterised by recurrent flares of generalised sterile pustules and associated systemic features. Inconsistent diagnostic criteria and a lack of approved therapies pose serious challenges to GPP management. Our objectives were to discuss the challenges encountered in the care of patients with GPP and identify healthcare provider (HCP) educational needs and clinical practice gaps in GPP management. METHODS: On 24 July 2020, 13 dermatologists from 10 countries (Brazil, Canada, China, Egypt, France, Germany, Japan, Malaysia, the UK and the USA) attended a workshop to share experiences in managing patients with GPP. Educational needs and clinical practice gaps grouped according to healthcare system level were discussed and ranked using interactive polling. RESULTS: Lack of experience of GPP among HCPs was identified as an important individual HCP-level clinical practice gap. Limited understanding of the presentation and pathogenesis of GPP among non-specialists means misdiagnosis is common, delaying referral and treatment. In countries where patients may present to general practitioners or emergency department HCPs, GPP is often mistaken for an infection. Among dermatologists who can accurately diagnose GPP, limited knowledge of treatments may necessitate referral to a colleague with more experience in GPP. At the organisational level, important needs identified were educating emergency department HCPs to recognise GPP as an autoinflammatory disease and improving communication, cooperation and definitions of roles within multidisciplinary teams supporting patients with GPP. At the regulatory level, robust clinical trial data, clear and consistent treatment guidelines and approved therapies were identified as high priorities. CONCLUSIONS: The educational imperative most consistently identified across the participating countries is for HCPs to understand that GPP can be life-threatening if appropriate treatment initiation is delayed, and to recognise when to refer patients to a colleague with more experience of GPP management. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s13555-021-00661-2. Springer Healthcare 2021-12-13 /pmc/articles/PMC8850517/ /pubmed/34904208 http://dx.doi.org/10.1007/s13555-021-00661-2 Text en © The Author(s) 2021, corrected publication 2022 https://creativecommons.org/licenses/by-nc/4.0/Open Access This article is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License, which permits any non-commercial use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) .
spellingShingle Original Research
Strober, Bruce
Leman, Joyce
Mockenhaupt, Maja
Nakano de Melo, Juliana
Nassar, Ahmed
Prajapati, Vimal H.
Romanelli, Paolo
Seneschal, Julien
Tsianakas, Athanasios
Wei, Lee Yoong
Yasuda, Masahito
Yu, Ning
Hernandez Daly, Ana C.
Okubo, Yukari
Unmet Educational Needs and Clinical Practice Gaps in the Management of Generalized Pustular Psoriasis: Global Perspectives from the Front Line
title Unmet Educational Needs and Clinical Practice Gaps in the Management of Generalized Pustular Psoriasis: Global Perspectives from the Front Line
title_full Unmet Educational Needs and Clinical Practice Gaps in the Management of Generalized Pustular Psoriasis: Global Perspectives from the Front Line
title_fullStr Unmet Educational Needs and Clinical Practice Gaps in the Management of Generalized Pustular Psoriasis: Global Perspectives from the Front Line
title_full_unstemmed Unmet Educational Needs and Clinical Practice Gaps in the Management of Generalized Pustular Psoriasis: Global Perspectives from the Front Line
title_short Unmet Educational Needs and Clinical Practice Gaps in the Management of Generalized Pustular Psoriasis: Global Perspectives from the Front Line
title_sort unmet educational needs and clinical practice gaps in the management of generalized pustular psoriasis: global perspectives from the front line
topic Original Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8850517/
https://www.ncbi.nlm.nih.gov/pubmed/34904208
http://dx.doi.org/10.1007/s13555-021-00661-2
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