Clinical and genetic differences between pustular psoriasis subtypes
Sophie Twelves,Alshimaa Mostafa,Alshimaa Mostafa,Nick Dand,Elias Burri,Katalin Farkas,Rosemary Wilson,Hywel L Cooper,Alan D. Irvine,Hazel H Oon,Külli Kingo,Sulev Kõks,Ulrich Mrowietz,Luis Puig,Nick J. Reynolds,Eugene Sern Ting Tan,Adrian Tanew,Kaspar Torz,Hannes Trattner,Mark C Valentine,Shyamal Wahie,Richard B. Warren,Andrew Wright,Zsuzsa Bata-Csörgő,Márta Széll,Christopher E.M. Griffiths,A. David Burden,Siew Eng Choon,Catherine H. Smith,Jonathan Barker,Alexander A. Navarini,Francesca Capon +31 more
TLDR
In this paper, the authors sought to characterize the clinical and genetic features of pustular psoriasis through the analysis of an extended patient cohort, including 863 unrelated patients (251 with GPP, 560 with PPP, 28 with acrodermatitis continua of Hallopeau [ACH], and 24 with multiple diagnoses).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 Conclusions The analysis of an unparalleled resource revealed key clinical and genetic differences between patients with PPP and those with GPP.read more
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Journal ArticleDOI
Pustular psoriasis with a focus on generalized pustular psoriasis: classification and diagnostic criteria. An Italian expert consensus
TL;DR: A review of the available diagnostic criteria for generalized pustular psoriasis (GPP) is presented in this paper , with the aim of promoting a better stratification and therapeutic management of this severe and heterogeneous disease.
Journal ArticleDOI
Pustular psoriasis with a focus on generalized pustular psoriasis: classification and diagnostic criteria. An Italian expert consensus.
Antonio Costanzo,Federico Bardazzi,P. De Simone,Gabriella Fabbrocini,C. Forti,Angelo V. Marzano,Luigi Naldi,Aurora Parodi,Francesca Prignano,Giampiero Girolomoni +9 more
TL;DR: In this article , a review of available diagnostic criteria for generalized pustular psoriasis (GPP) is presented, with the aim to promote a better stratification and therapeutic management of this severe and heterogeneous disease.
Journal ArticleDOI
Inpatient Management of Psoriasis: A Current Perspective and Update for Clinicians
Edward Hadeler,Megan Mosca,Julie Hong,Nicholas Brownstone,Wilson Liao,Tina Bhutani,Kanade Shinkai +6 more
TL;DR: In this article, the authors describe the current literature on the psoriasis inpatient population, discuss considerations for admission, and provide updated recommendations for inpatient psoriatic workup, management, and post-discharge follow-up.
Book ChapterDOI
Challenges in Psoriasis Treatment: Nail, Scalp, and Palmoplantar Involvement
TL;DR: The challenges posed by each condition and evaluate available treatment options are discussed in this article, where the authors critically address the challenges posed for each condition, evaluate the treatment options, and evaluate the available treatment strategies.
Journal ArticleDOI
Treatment of Palmoplantar Pustulosis Using Fixed Combination Halobetasol Propionate 0.01% and Tazarotene 0.045% Lotion: A Case Report:
TL;DR: A 54-year-old woman who presented with a painful pustular lesion on her right palm was diagnosed with palmoplantar pustulosis and switched to halobetasol propionate 0.01% and tazarotene 0.045% lotion, a novel corticosteroid–retinoid combination, while awaiting payor approval for systemic biologic therapy.
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Interleukin-36–Receptor Antagonist Deficiency and Generalized Pustular Psoriasis
Slaheddine Marrakchi,Philippe Guigue,Blair R. Renshaw,Anne Puel,Xue-Yuan Pei,Sylvie Fraitag,Jihen Zribi,Elodie Bal,Céline Cluzeau,Maya Chrabieh,Jennifer E. Towne,Jason Douangpanya,Christian Pons,Sourour Mansour,Valérie Serre,Hafedh Makni,Nadia Mahfoudh,Faiza Fakhfakh,Christine Bodemer,Josué Feingold,Smail Hadj-Rabia,Michel Favre,Emmanuelle Génin,Mourad Sahbatou,Arnold Munnich,Jean-Laurent Casanova,John E. Sims,Hamida Turki,Hervé Bachelez,Asma Smahi +29 more
TL;DR: Aberrant interleukin-36Ra structure and function lead to unregulated secretion of inflammatory cytokines and generalized pustular psoriasis.
Journal ArticleDOI
Mutations in IL36RN/IL1F5 are associated with the severe episodic inflammatory skin disease known as generalized pustular psoriasis
Alexandros Onoufriadis,Michael A. Simpson,Andrew Pink,Paola Di Meglio,Catherine H. Smith,Venu Pullabhatla,Jo Knight,Sarah L. Spain,Frank O. Nestle,A. David Burden,Francesca Capon,Richard C. Trembath,Jonathan Barker +12 more
TL;DR: Findings suggest loss of function of IL36RN as the genetic basis of GPP and implicate innate immune dysregulation in this severe episodic inflammatory disease, thereby highlighting IL-1 signaling as a potential target for therapeutic intervention.
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