Generalized pustular psoriasis : A global Delphi consensus on clinical course, diagnosis, treatment goals and disease management
Puig Sanz, Lluís (Institut d'Investigació Biomèdica Sant Pau)
Choon, Siew Eng (Monash University)
Gottlieb, Alice B. (Icahn School of Medicine at Mount Sinai (Nova York, Estats Units d'Amèrica))
Marrakchi, Slaheddine (University of Sfax)
Prinz, Jörg C. (Ludwig-Maximilian-University Munich)
Romiti, Ricardo (Universidade de São Paulo)
Tada, Yayoi (Teikyo University School of Medicine)
von Bredow, Dorothea (IQVIA Commercial GmbH & Co.)
Gooderham, Melinda (Queen's University and Centre for Dermatology and Probity Medical Research)
Universitat Autònoma de Barcelona

Date:	2023
Abstract:	Generalized 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. Conduct a Delphi panel study to identify current evidence and gain advanced insights into GPP. A 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%. Twenty-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. Global 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.
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, i la comunicació pública de l'obra, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original. No es permet la creació d'obres derivades.
Language:	Anglès
Document:	Article ; recerca ; Versió publicada
Subject:	Consensus ; Delphi Technique ; Disease Management ; Disease Progression ; Goals ; Humans ; Psoriasis
Published in:	Journal of the European Academy of Dermatology and Venereology, Vol. 37 Núm. 4 (april 2023) , p. 737-752, ISSN 1468-3083

DOI: 10.1111/jdv.18851
PMID: 36606566

16 p, 4.2 MB

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Research literature > UAB research groups literature > Research Centres and Groups (research output) > Health sciences and biosciences > Institut de Recerca Sant Pau
Articles > Research articles
Articles > Published articles