Generalized pustular psoriasis: A global Delphi consensus on clinical course, diagnosis, treatment goals and disease management

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art_PUIG_Generalized_pustular_psoriasis_A_global_Delphi_consensus_on_2023.PDF (4.2 MB)
Citações na Scopus
14
Tipo de produção
article
Data de publicação
2023
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
WILEY
Autores
PUIG, Lluis
CHOON, Siew Eng
GOTTLIEB, Alice B. B.
MARRAKCHI, Slaheddine
PRINZ, Joerg C.
TADA, Yayoi
BREDOW, Dorothea von
GOODERHAM, Melinda
Citação
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY, v.37, n.4, p.737-752, 2023
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
BackgroundGeneralized pustular psoriasis (GPP) is a rare and highly heterogeneous skin disease, characterized by flares of neutrophilic pustules and erythema. As a rare disease with few clinical studies and no standardized management approaches, there is a paucity of knowledge regarding GPP. ObjectivesConduct a Delphi panel study to identify current evidence and gain advanced insights into GPP. MethodsA systematic literature review was used to identify published literature and develop statements categorized into four key domains: clinical course and flare definition; diagnosis; treatment goals; and holistic management. Statements were rated on a Likert scale by a panel of dermatologists in two rounds of online questionnaires; the threshold for consensus was agreement by >= 80%. ResultsTwenty-one panellists reached consensus on 70.9%, 61.8%, 100.0% and 81.8% of statements in the 'clinical course and flare definition', 'diagnosis', 'treatment goals' and 'holistic management of GPP' domains, respectively. There was clear consensus on GPP being phenotypically, genetically and immunologically distinct from plaque psoriasis. Clinical course is highly variable, with an extensive range of complications. Clinical and histologic features supporting GPP diagnosis reached high levels of agreement, and although laboratory evaluations were considered helpful for diagnosis and monitoring disease severity, there was uncertainty around the value of individual tests. All acute and long-term treatment goals reached consensus, including rapid and sustained clearance of pustules, erythema, scaling and crust, clearance of skin lesions and prevention of new flares. Potential triggers, associated comorbidities and differential diagnoses achieved low rates of consensus, indicating that further evidence is needed. ConclusionsGlobal consensus between dermatologists was reached on clinically meaningful goals for GPP treatment, on key features of GPP flares and on approaches for assessing disease severity and multidisciplinary management of patients. On this basis, we present a management algorithm for patients with GPP for use in clinical practice.
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URI
https://observatorio.fm.usp.br/handle/OPI/54201
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Coleções
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - LIM/50
Artigos e Materiais de Revistas Científicas - LIM/53