Hsu Francis Li-Tien, Tsai Tsen-Fang
Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan.
Department of Dermatology, National Taiwan University College of Medicine, Taipei, Taiwan.
Am J Clin Dermatol. 2025 May;26(3):395-409. doi: 10.1007/s40257-025-00937-9. Epub 2025 Apr 1.
Ethnic differences of the clinicopathological characteristics in many immune-mediated skin diseases have been reported, including psoriasis vulgaris (PV). However, the ethnic differences of pustular psoriasis have been less studied.
The aim of this study was to compare the differences in epidemiology, genetic background, clinical manifestations, treatment patterns and responses among Asian and non-Asian patients with pustular psoriasis, including generalized pustular psoriasis (GPP) and acrodermatitis continua of Hallopeau (ACH).
This systematic review was based on a comprehensive search of Cochrane, PubMed, and Embase databases from earliest available date to 31 December 2024, and all studies reporting on patients with either GPP or ACH irrespective of study design. Studies with study size below five patients or those focusing on quality of life or economic aspects were excluded. In each publication, the ethnic composition, demographics information, disease course and manifestation, as well as genetic mutations, treatment type and response were collected if available.
Of 2187 screened studies, 141 studies were included, with the majority being cohort studies. Compared with other ethnicities, East Asians with GPP carried more null IL36RN mutations, while AP1S3 mutations seemed absent in Asians. Phenotypically, Asians had younger onset age, bimodal age distribution, less family history of PV, and more scalp/nail involvement. In Asians, absence of coexisting PV was associated with severe disease. GPP with PV had shorter pre-pustular duration among Asians than non-Asians. Use of acitretin appeared higher and more effective among East Asians compared with other populations. In ACH, Asians mostly carried homozygous null IL36RN mutations and had younger onset age, more multi-digit involvement, persistent treatment course, and more coexisting GPP than Europeans. Biologics use was less common in Asia in both GPP and ACH than in Europe and the US.
This systematic review underscores notable ethnic differences in genetic profiles, clinical features, and therapeutic responses in GPP and ACH. The diagnosis of GPP and ACH may differ across studies and the true impacts of ethnicities on these differences remain to be confirmed. Nonetheless, the results from this study enhance our understanding of the heterogeneous characteristics of GPP and ACH, highlighting the necessity of incorporating ethnic differences into the diagnosis, genetic testing, and management strategies for patients with GPP and ACH.
已有报道称许多免疫介导性皮肤病的临床病理特征存在种族差异，包括寻常型银屑病（PV）。然而，脓疱型银屑病的种族差异研究较少。
本研究旨在比较亚洲和非亚洲脓疱型银屑病患者（包括泛发性脓疱型银屑病（GPP）和Hallopeau连续性肢端皮炎（ACH））在流行病学、遗传背景、临床表现、治疗模式及反应方面的差异。
本系统评价基于对Cochrane、PubMed和Embase数据库从最早可用日期至2024年12月31日的全面检索，纳入所有报道GPP或ACH患者的研究，无论研究设计如何。排除研究样本量少于5例患者的研究，以及关注生活质量或经济方面的研究。在每篇出版物中，如有可用信息，则收集种族构成、人口统计学信息、病程和表现，以及基因突变、治疗类型和反应。
在筛选的2187项研究中，纳入了141项研究，其中大多数为队列研究。与其他种族相比，GPP东亚患者携带更多IL36RN基因无效突变，而亚洲人似乎不存在AP1S3突变。从表型上看，亚洲人发病年龄更小，年龄分布呈双峰型，PV家族史较少，头皮/指甲受累更多。在亚洲人中，不存在共存的PV与严重疾病相关。与非亚洲人相比，亚洲人GPP合并PV时脓疱前期持续时间更短。与其他人群相比，阿维A在东亚人中的使用似乎更高且更有效。在ACH中，亚洲人大多携带纯合IL36RN基因无效突变，发病年龄更小，多指受累更多，治疗过程持续，且比欧洲人共存GPP更多。在GPP和ACH中，亚洲地区生物制剂的使用均不如欧洲和美国普遍。
本系统评价强调了GPP和ACH在基因谱、临床特征和治疗反应方面存在显著的种族差异。不同研究中GPP和ACH的诊断可能存在差异，种族对这些差异的真正影响仍有待证实。尽管如此，本研究结果增进了我们对GPP和ACH异质性特征的理解，突出了将种族差异纳入GPP和ACH患者诊断、基因检测及管理策略的必要性。
