European consensus statement on phenotypes of pustular psoriasis

A. A. Navarini*, A. D. Burden, F. Capon, U. Mrowietz, L. Puig, S. Köks, K. Kingo, C. Smith, J. N. Barker, Hervé Bachelez, Andrea Chiricozzi, Antonio Costanzo, Kilian Eyerich, Lars E. French, Kamran Ghoreschi, Michel Gilliet, Giampiero Girolomoni, Robert Gniadecki, Christopher Griffiths, Hong Yi KohDan Lipsker, Luigi Naldi, Ele Prans, Jörg Prinz, Kristian Reich, Martin Röcken, Lone Skov, George Sorin, Mona Ståhle, Georg Stingl, Peter Van de Kerkhof, Richard Warren

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

282 Citations (Scopus)


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 [Saurat Dermatologie 2016, Rook's Dermatology 2016, Fitzpatrick's 2012 and Braun-Falco 2012], 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.

Original languageEnglish
Pages (from-to)1792-1799
Number of pages8
JournalJournal of The European Academy of Dermatology and Venereology
Issue number11
Publication statusPublished - 15 Nov 2017


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