Prevalence, comorbidities and mortality of generalized pustular psoriasis: A literature review

Abstract

Generalized pustular psoriasis (GPP) is a rare auto‐inflammatory skin disease characterised by acute episodes of sterile pustule formation. Diagnosis and treatment of the disease have historically been complicated by a lack of awareness, and no consistent global definition or clinical coding standards. Now acknowledged as a distinct clinical entity with a recognised genetic component, GPP can take a serious and life‐threatening course due to systemic inflammatory complications and its association with various comorbidities. As with other rare diseases, there are significant challenges to understanding the epidemiology of GPP, notably a small patient population, non‐standardised study methodologies and ethnic differences in its presentation. A clearer understanding of GPP is therefore required for clinicians to better manage patients with this rare condition. In this review article, we present an overview of the available data on GPP prevalence estimates in key demographics and report the frequency of genetic mutations associated with the disease. We detail the incidence of known comorbidities and summarise the data on mortality and assigned causes of death. Lastly, we discuss the various factors that impact the collection, interpretation and comparison of these data.

Current developments and perspectives in psoriasis

“…Transcriptome comparisons with healthy skin demonstrate aberrant expression of high triple‐digit numbers of gene products in both psoriasis vulgaris and pustular psoriasis (with rather little overlap) 54,55 . The involvement of other organ systems (comorbidity) and provocation factors also influence the course of psoriasis as a systemic disease 56–59 …”

“…54,55 The involvement of other organ systems (comorbidity) and provocation factors also influence the course of psoriasis as a systemic disease. [56][57][58][59] From these brief descriptions, it is clear that the pathophysiology of psoriasis is complex. It is becoming increasingly clear that the overall pattern of inflammatory mediators and cells, which may well shift in the (lifelong) course of the disease, is ultimately responsible for the individual manifestation.…”

Aktuelle Entwicklungen und Perspektiven bei Psoriasis

“…54,55 Auch die Beteiligung anderer Organsysteme (Komorbidität) und Provokationsfaktoren beeinflussen den Verlauf der Psoriasis als Systemerkrankung. [56][57][58][59] Anhand dieser streiflichtartigen Ausführungen wird bereits klar, dass die Pathophysiologie der Psoriasis komplex ist. Zunehmend wird deutlich, dass das Gesamtmuster der Entzündungsmediatoren und -zellen, welches sich im Laufe der "Krankheitskarriere" durchaus verschieben kann, letztlich für die individuelle Manifestation verantwortlich ist.…”

“…Transkriptomanalysen im Vergleich zu gesunder Haut belegen die aberrante Expression hoher dreistelliger Zahlen von Genprodukten sowohl bei Psoriasis vulgaris als auch bei pustulöser Psoriasis (wobei die Überlappung relativ gering ist) 54,55 . Auch die Beteiligung anderer Organsysteme (Komorbidität) und Provokationsfaktoren beeinflussen den Verlauf der Psoriasis als Systemerkrankung 56–59 …”

A Familial Novel Putative-Pathogenic Mutation Identified in Plaque-Psoriasis by a Multigene Panel Analysis

“…Psoriasis has been primarily defined as an autoimmune, T-cell-mediated disease with a dysregulated inflammatory response that is composed of both innate and adaptive immunity [1,2]. Although it is preferentially a skin disease, recent studies highlight the association with several different multiorgan comorbidities implicated in the systemic inflammation [3][4][5][6]. Advances in molecular and cellular research have enabled the immunological features of psoriasis to be deciphered and helped to classify this chronic inflammatory skin disorder as the prototypic IL-23 and IL-17-dominated disease in humans [7].…”