The clinical, humanistic, and economic burden of generalized pustular psoriasis: a structured review

Abstract

Introduction: Generalized pustular psoriasis (GPP) is characterized by widespread erythema and edema, superficial sterile coalescing pustules, and lakes of pus. Although the impact of GPP is thought to be substantial, emerging literature on its clinical, humanistic, and economic burden has not previously been described in a structured way. Areas covered: A structured search focused on the identification of studies in GPP using specific search terms in PubMed and EMBASE® from 2005 onwards, with additional back-referencing and pragmatic searches. Outcomes of interest included clinical, humanistic, and economic burden. Expert opinion: Despite its significant clinical, humanistic, and economic burden, GPP is poorly classified and inadequately studied. A recent European (ERASPEN) consensus classifies GPP into relapsing and persistent disease and classifies patients on the presence or absence of psoriasis vulgaris. Classification of GPP lesions involving >30% body surface area or use of hospitalization as a surrogate may be a way to identify significant flares. Given the frequency of flares, the impaired quality of life during the post-flare period, and safety/tolerability issues, it is clear that current treatment options are not sufficient. Long-term studies utilizing the European consensus statement with subclassifiers are required to supplement our current understanding of the burden of GPP.

Drug survival in the treatment of generalized pustular psoriasis: A retrospective multicenter study

“…We found a female predominance with a ratio of approximately 3:2 which was also reported by Baker and Ryan 25 and in the European ERASPEN cohort (female proportion: 62.5%) 26 . The mean age at symptom onset was 51.2 years, comparable to reported onset of disease in the fifth decade of life 25,27,28 . In our cohort, infections, withdrawal of antipsoriatic medication, particularly systemic glucocorticoids, and intake of new medication were suspected trigger factors of GPP manifestation by the treating physicians, consistent with previous reports 1,8 .…”

“…26 The mean age at symptom onset was 51.2 years, comparable to reported onset of disease in the fifth decade of life. 25,27,28 In our cohort, infections, withdrawal of antipsoriatic medication, particularly systemic glucocorticoids, and intake of new medication were suspected trigger factors of GPP manifestation by the treating physicians, consistent with previous reports. 1,8 According to diagnostic criteria, persistence of pustular rash for at least 3 months or at least one relapse is required for a definite diagnosis of GPP.…”

Characteristics of hospitalizations and emergency department visits due to generalized pustular psoriasis in the United States

“…GPP is characterized by rapidly progressing diffuse dark erythematous patches with pustules that may coalesce to form lakes of pus distributed across the body 3 . The skin is often painful and tender causing patient quality of life to be severely impaired during the course of a GPP flare 4,5 . Little is known about the prevalence or the incidence of GPP and published estimates range from 0.2 and 0.7 per 100,000 persons from separate studies in France and Japan, respectively 2,6 .…”

The clinical, humanistic, and economic burden of palmoplantar pustulosis: a structured review

“…Pustular psoriasis (PP) describes 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 [1]. As described in the sister publication to this review [2], recent classification of pustular psoriasis proposed by the European Rare and Severe Expert Network (ERASPEN) notes the distinction between psoriasis vulgaris (PV) and PP and recognizes them as distinct phenotypes [1].…”