European consensus statement on phenotypes of pustular psoriasis

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 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.

Generalized pustular psoriasis – a model disease for specific targeted immunotherapy, systematic review

“…Thus, compared to psoriasis with an estimated prevalence varying between 0.9% and 8.5%, GPP is a rather rare disease and some experts regard it as an infrequent variant of psoriasis. It is discussed controversially whether GPP should be considered a distinct clinical entity rather than a variant of psoriasis . In a Japanese epidemiological analysis of 9339 psoriatic patients, GPP was found to represent 1.3% of all clinical types of psoriasis .…”

Generalized pustular psoriasis: a review and update on treatment

“…Patients are visibly ill, with high‐grade fever, malaise, neutrophilic leucocytosis, elevated C‐reactive protein levels and peripheral eosinophilia . The pustules occur at the edges of expanding erythematous plaques or over erythematous skin . While the cutaneous manifestation is the most striking observation, extracutaneous involvement warrants hospital admission and immediate treatment.…”

Treatment of pustular psoriasis with anakinra: a statistical analysis plan for stage 1 of an adaptive two-staged randomised placebo-controlled trial

“…Palmoplantar pustulosis (PPP) is a rare pustular form of the disease which affects the hands and feet and is characterised by painful, intensely inflamed, red skin studded by sheets of monomorphic, sterile, neutrophilic pustules [1, 2]. It is known to affect quality of life at a level comparable to that from major medical and psychiatric illness, yet treatment options for PPP are profoundly limited [3].…”