European consensus statement on phenotypes of pustular psoriasis

Navarini, A. A., Burden, A. D., Capon, F., Mrowietz, U., Puig, L., Köks, S., Kingo, K., Smith, C. and Barker, J. N. (2017) European consensus statement on phenotypes of pustular psoriasis. Journal of the European Academy of Dermatology and Venereology, 31(11), pp. 1792-1799. (doi: 10.1111/jdv.14386) (PMID:28585342)

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Abstract

Pustular psoriasis (PP) is a group of inflammatory skin conditions characterized by infiltration of neutrophil granulocytes in the epidermis to such an extent that clinically visible sterile pustules develop. Because of clinical co-incidence, PP is currently grouped with psoriasis vulgaris (PV). However, PP and PV are phenotypically different, respond differently to treatments, and seem to be distinct on the genetic level. In contrast to PV, the phenotypes of PP are not well defined. Descriptions of each form of PP are discordant among standard dermatology textbooks [1-5], encumbering the collection of phenotypically well-matched groups of patients as well as clinical trials. The European Rare and Severe Psoriasis Expert Network (ERASPEN) was founded to define consensus criteria for diagnosis, deeply phenotype large groups of PP patients, analyse the genetics and pathophysiology and prepare for prospective clinical trials. This work reviews historical aspects of these conditions, new genetic findings and presents our initial considerations on the phenotypes of PP and a consensus classification of clinical phenotypes that will be used as a baseline for further, prospective studies of PP. Generalized Pustular Psoriasis (GPP) is defined as primary, sterile, macroscopically visible pustules on non-acral skin (excluding cases where pustulation is restricted to psoriatic plaques). GPP can occur with or without systemic inflammation, with or without PV and can either be a relapsing (>1 episode) or persistent (> 3 months) condition. Acrodermatitis continua of Hallopeau (ACH) is characterized by primary, persistent (> 3 months), sterile, macroscopically visible pustules affecting the nail apparatus. Palmoplantar pustulosis (PPP) has primary, persistent (> 3 months), sterile, macroscopically visible pustules on palms and/or soles and can occur with or without PV.

Item Type:Articles
Additional Information:ERASPEN is supported by a PPRC grant from the European Association of Dermatology and Venereology (EADV) to AN and JB. AN is supported by the Bruno Bloch and Promedica Foundation and HSM-2 from the Kanton of Zurich, Switzerland. FC is supported by The Psoriasis Association.
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Burden, Professor David
Authors: Navarini, A. A., Burden, A. D., Capon, F., Mrowietz, U., Puig, L., Köks, S., Kingo, K., Smith, C., and Barker, J. N.
College/School:College of Medical Veterinary and Life Sciences > Institute of Infection Immunity and Inflammation
Journal Name:Journal of the European Academy of Dermatology and Venereology
Publisher:Wiley
ISSN:0926-9959
ISSN (Online):1468-3083
Published Online:06 June 2017
Copyright Holders:Copyright © 2017 European Academy of Dermatology and Venereology
First Published:First published in Journal of the European Academy of Dermatology and Venereology 31(11): 1792-1799
Publisher Policy:Reproduced in accordance with the copyright policy of the publisher

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