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Journal article
Clinical and genetic differences between pustular psoriasis subtypes
Journal of allergy and clinical immunology, Vol.143(3), pp.1021-1026
2019
PMCID: PMC6403101
PMID: 30036598
Abstract
Background: The term pustular psoriasis indicates a group of severe skin disorders characterized by eruptions of neutrophil-filled pustules. The disease, which often manifests with concurrent psoriasis vulgaris, can have an acute systemic (generalized pustular psoriasis [GPP]) or chronic localized (palmoplantar pustulosis [PPP] and acrodermatitis continua of Hallopeau [ACH]) presentation. Although mutations have been uncovered in IL36RN and AP1S3, the rarity of the disease has hindered the study of genotype-phenotype correlations.
Objective: We sought to characterize the clinical and genetic features of pustular psoriasis through the analysis of an extended patient cohort.
Methods: We ascertained a data set of unprecedented size, including 863 unrelated patients (251 with GPP, 560 with PPP, 28 with ACH, and 24 with multiple diagnoses). We undertook mutation screening in 473 cases.
Results: Psoriasis vulgaris concurrence was lowest in PPP (15.8% vs 54.4% in GPP and 46.2% in ACH, P<.0005 for both), whereas themean age of onset was earliest in GPP (31.0 vs 43.7 years in PPP and 51.8 years inACH, P<.0001 for both). The percentage of female patients was greater in PPP (77.0%) than in GPP (62.5%; P = 5.8 x 10(-5)). The same applied to the prevalence of smokers (79.8% vs 28.3%, P < 10(-1)5). Although AP1S3 alleles had similar frequency (0.03-0.05) across disease subtypes, IL36RN mutations were less common in patients with PPP (0.03) than in those with GPP (0.19) andACH(0.16; P51.9310 214 and.002, respectively). Importantly, IL36RN disease alleles had a dose-dependent effect on age of onset in all forms of pustular psoriasis (P = .003).
Conclusions: The analysis of an unparalleled resource revealed key clinical and genetic differences between patients with PPP and those with GPP.
Details
- Title
- Clinical and genetic differences between pustular psoriasis subtypes
- Authors/Creators
- Sophie Twelves - King's College LondonAlshimaa Mostafa - University Hospital of ZurichNick Dand - King's College LondonElias Burri - University Hospital of ZurichKatalin Farkas - University of SzegedRosemary Wilson - Supreme Council Of HealthHywel L. Cooper - Portsmouth Hospitals NHS TrustAlan D. Irvine - Our Lady's HospitalHazel H. Oon - National Skin CentreKulli Kingo - Tartu University HospitalSulev Koks - University of TartuUlrich Mrowietz - Clinical Research Center KielLuis Puig - Hosp Santa Creu & Sant Pau, Dept Dermatol, Barcelona, SpainNick Reynolds - Newcastle upon Tyne Hospitals NHS Foundation TrustEugene Sern-Ting Tan - Natl Skin Ctr, Dept Dermatol, Singapore, SingaporeAdrian Tanew - Medical University of ViennaKaspar Torz - Clinical Research Center KielHannes Trattner - Medical University of ViennaMark Valentine - University of WashingtonShyamal Wahie - Darlington Memorial HospitalRichard B. Warren - Manchester Academic Health Science CentreAndrew Wright - St. Luke's HospitalZsuzsa Bata-Csorgo - MTA SZTE Dermatol Res Grp, Szeged, HungaryMarta Szell - University of SzegedChristopher E. M. Griffiths - Manchester Academic Health Science CentreA. David Burden - University of GlasgowSiew-Eng Choon - Sultanah Aminah HospitalCatherine H. Smith - King's College LondonJonathan N. Barker - King's College LondonAlexander A. Navarini - University Hospital of ZurichFrancesca Capon - King's College London
- Publication Details
- Journal of allergy and clinical immunology, Vol.143(3), pp.1021-1026
- Publisher
- Elsevier Inc. on behalf of the American Academy of Allergy, Asthma & Immunology.
- Number of pages
- 6
- Grant note
- guysbrc-2012-1; EME/13/50/17 / National Institutes of Health Research (NIHR) guysbrc-2012-1 / King's College Hospital NHS Foundation Trust Department of Health through the National Institute for Health Research (NIHR) BioResource Clinical Research Facility and comprehensive Biomedical Research Centre Guy's and St Thomas' Charity Prize PhD Programme in Biomedical and Translational Science King's College London MRC; UK Research & Innovation (UKRI); Medical Research Council UK (MRC) Efficacy and Mechanism Evaluation (EME) Programme King's Bioscience Institute EME 13/50/17 / NIHR; National Institutes of Health Research (NIHR) PPRC grant from the European Association of Dermatology and Venereology (EADV) MR/L011808/1 / Medical Research Council (MRC) Stratified Medicine award; UK Research & Innovation (UKRI); Medical Research Council UK (MRC) Newcastle MRC/EPSRC Molecular Pathology Node; UK Research & Innovation (UKRI); Engineering & Physical Sciences Research Council (EPSRC); Medical Research Council UK (MRC) MR/S003126/1; MR/N005872/1; MR/L011808/1 / MRC; UK Research & Innovation (UKRI); Medical Research Council UK (MRC)
- Identifiers
- 991005634027507891
- Copyright
- © 2018 The Authors.
- Murdoch Affiliation
- Personalised Medicine Centre
- Language
- English
- Resource Type
- Journal article
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- Collaboration types
- Domestic collaboration
- International collaboration
- Citation topics
- 1 Clinical & Life Sciences
- 1.106 Rheumatology
- 1.106.737 Psoriasis
- Web Of Science research areas
- Allergy
- Immunology
- ESI research areas
- Immunology