Affiliations 

  • 1 Department of Medical and Molecular Genetics, School of Basic and Medical Biosciences, King's College London, London, United Kingdom
  • 2 Department of Dermatology, University Hospital Zurich, Zurich, Switzerland; Department of Dermatology, Beni Suef University, Beni Suef, Egypt
  • 3 Department of Dermatology, University Hospital Zurich, Zurich, Switzerland
  • 4 Department of Medical Genetics, University of Szeged, Szeged, Hungary
  • 5 St John's Institute of Dermatology, School of Basic and Medical Biosciences, King's College London, London, United Kingdom
  • 6 Portsmouth Dermatology Unit, Portsmouth Hospitals Trust, Portsmouth, United Kingdom
  • 7 Paediatric Dermatology, Our Lady's Children's Hospital Crumlin, and Clinical Medicine, Trinity College Dublin, Dublin, Ireland
  • 8 Department of Dermatology, National Skin Centre, Singapore
  • 9 Department of Dermatology, University of Tartu, and the Clinic of Dermatology, Tartu University Hospital, Tartu, Estonia
  • 10 Department of Pathophysiology, University of Tartu, Tartu, Estonia
  • 11 Psoriasis Center at the Department of Dermatology, University Medical Center, Schleswig-Holstein, Campus Kiel, Kiel, Germany
  • 12 Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
  • 13 Institute of Cellular Medicine, Medical School, Newcastle University and the Department of Dermatology, Royal Victoria Infirmary, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom
  • 14 Department of Dermatology, Medical University of Vienna, Vienna, Austria
  • 15 Division of Dermatology, University of Washington School of Medicine, Seattle, Wash
  • 16 University Hospital of North Durham and Darlington Memorial Hospital, Darlington, United Kingdom
  • 17 Dermatology Centre, Salford Royal Hospital, University of Manchester and the Academic Health Science Centre, Manchester, United Kingdom
  • 18 St Lukes Hospital, Bradford, and the Centre for Skin Science, University of Bradford, Bradford, United Kingdom
  • 19 MTA-SZTE Dermatological Research Group, Szeged, and the Department of Dermatology and Allergology, University of Szeged, Szeged, Hungary
  • 20 MTA-SZTE Dermatological Research Group, Szeged, and the Department of Medical Genetics, University of Szeged, Szeged, Hungary
  • 21 Institute of Infection, Inflammation and Immunity, University of Glasgow, Glasgow, United Kingdom
  • 22 Department of Dermatology, Hospital Sultanah Aminah, Jeffrey Cheah School of Medicine and Health Sciences, Monash University Malaysia, Johor Bahru, Selangor, Malaysia
  • 23 St John's Institute of Dermatology, School of Basic and Medical Biosciences, King's College London, London, United Kingdom. Electronic address: jonathan.barker@kcl.ac.uk
PMID: 30036598 DOI: 10.1016/j.jaci.2018.06.038

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 

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.