广泛化脓疱性银屑病（GPP）是一种罕见、严重的脓疱性银屑病。它的定义是，在非肢端皮肤上持续出现或复发的肉眼可见的无菌原发性脓疱，而不是在银屑病斑块内。由于其罕见性，缺乏关于GPP及其相关胃肠道（GI）和肝脏疾病的随机对照试验。本文概述了与GPP相关的GI和肝脏疾病。已知GPP与中性粒细胞性胆管炎等皮肤外表现有关。90%的GPP患者被诊断时报告肝功能异常。较少见的是，胰腺炎和胃肠道出血被归因于GPP。虽然一个包含7.5%脓疱性银屑病患者的银屑病登记表报告了与乙型和丙型病毒性肝炎的关联，但真正的关系仍需进一步阐明，因为乙肝在亚洲是流行的，GPP的患病率也更高。已经确定了GPP和肝细胞癌和炎症性肠病等情况之间的常见基因突变，解释了它们可能的关联，并提供了这些情况潜在治疗选项的答案。不了解这些关联可能导致对治疗GPP的有效和确定性药物进行不必要的撤回。了解相关GI和肝脏疾病的特征对于针对适当治疗的重要影响具有重要意义。 © 2023 John Wiley＆Sons A / S。John Wiley＆Sons Ltd.发布。

Generalised pustular psoriasis (GPP) is a rare and severe form of pustular psoriasis. It is defined by persisting or relapsing macroscopically visible sterile primary pustules occurring on non-acral skin and not within psoriasis plaques. Due to its rarity, there is a lack of randomised controlled trials on GPP and its associated gastrointestinal (GI) and liver disorders. In this article, we present a review of the GI and hepatic disorders associated with GPP. GPP is known to be associated with extracutaneous manifestations such as neutrophilic cholangitis. Abnormal liver function tests are reported in up to 90% of patients with GPP upon diagnosis. Less commonly, pancreatitis and gastrointestinal bleeding have been attributed to GPP. While a psoriasis registry with 7.5% prevalence of pustular psoriasis reported an association with viral hepatitis B and C, the true relationship remains to be elucidated as hepatitis B is endemic in Asia where GPP prevalence is higher. Common genetic mutations between GPP and conditions such as hepatocellular carcinoma and inflammatory bowel disease have been identified, explaining their possible associations and providing answers to potential therapeutic options for these conditions. A lack of recognition of these association may result in unnecessary withdrawal of efficacious and definitive drugs for the treatment of GPP. Understanding the characteristics of the associated GI and hepatic disorders will have important implications for targeting the appropriate therapeutics.© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.