Gastrointestinal and hepatic manifestations in patients with generalised pustular psoriasis

Abstract

Generalised pustular psoriasis is a rare and severe form of pustular psoriasis. It was first described by Leopold von Zumbusch in 1910. 1 In 2017, The European Rare and Severe Psoriasis Expert Network (ERASPEN) classified pustular psoriasis into three phenotypes, namely generalised pustular psoriasis (GPP), acrodermatitis continua of Hallopeau and palmoplantar pustulosis. 2 GPP is defined by persisting or relapsing macroscopically visible sterile primary pustules occurring on non-acral skin and not within psoriasis plaques. The Japanese guidelines in 2018 diagnose GPP based on four primary parameters: (a) systemic symptoms such as fever and fatigue; (b) systemic or extensive flush accompanied by multiple sterile pustules that sometimes merge to form lakes of pus; (c) neutrophilic subcorneal pustules histopathologically characterised by Kogoj's spongiform pustules; and (d) recurrence of the described clinical and histological features. 3 A definite diagnosis of GPP is made with the presence of all four criteria.The prevalence of GPP is higher in Asians (7.46 per million in Japan) 4 compared to the Caucasian population (1.76 per million in France 5 and 3.8 per 100 000 in Sweden). 6 There is a female preponderance for GPP and the mean age at onset of GPP was 40.9 years. 7

Understanding generalized pustular psoriasis for improved patient care

“…This special issue also highlights the extracutaneous manifestations of GPP, with Lim et al focusing on gastrointestinal and hepatic involvement 5 . Understanding the extracutaneous manifestations of GPP can guide clinicians in providing comprehensive care to patients and managing potential comorbidities effectively.…”