Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Division of Dermatology, Department of Medicine, Chiangrai Prachanukroh Hospital, Phayao, Thailand.
Exp Dermatol. 2023 Aug;32(8):1235-1245. doi: 10.1111/exd.14805. Epub 2023 Apr 14.
Pustular psoriasis is characterised by eruptions of neutrophilic sterile pustules. The European Rare and Severe Psoriasis Expert Network consensus defines pustular psoriasis into three subtypes; generalised pustular psoriasis (GPP), palmoplantar pustulosis and acrodermatitis continua of Hallopeau (ACH). Mixed forms are categorised according to their predominant features. However, the Japanese Dermatological Association includes ACH under the diagnosis of GPP. This article aims to review the similarities and differences between ACH and GPP. Based on our review, interleukin (IL)-36RN mutations, the most frequent genetic findings in pustular psoriasis are found most commonly in GPP, followed by ACH. Genotypes of IL-36RN mutations among GPP patients and ACH patients are different between European and Asian ethnicities. IL-36 signalling pathway is the main mechanism. Metabolic diseases are common comorbidities and joint involvement can occur in 20.5%-36.4% of both conditions. Associated plaque psoriasis is more common in GPP than in ACH. Generally, ACH, even the generalised type, does not have systemic inflammation whereas GPP can occur with or without systemic inflammation. ACH can occur before, simultaneously, or after the development of GPP. However, response to treatment for GPP and ACH even in the same patients appear to be different. ACH seemed to be more recalcitrant to treatment than GPP but severe flare of GPP can lead to morbidity and mortality. Although GPP and ACH share genotypes and pathogenesis, we believe that ACH should be classified separately from GPP, and not under diagnosis of GPP. Future research is warranted to satisfactorily distinguish the two conditions.
脓疱型银屑病的特征是中性粒细胞性无菌脓疱的发作。欧洲罕见和严重银屑病专家网络共识将脓疱型银屑病分为三种亚型; 泛发性脓疱型银屑病 (GPP)、掌跖脓疱病和 Hallopeau 连续性肢端皮炎 (ACH)。混合形式根据其主要特征进行分类。然而，日本皮肤病协会将 ACH 纳入 GPP 的诊断。本文旨在综述 ACH 和 GPP 之间的异同。基于我们的综述，白细胞介素 (IL)-36RN 突变是脓疱型银屑病最常见的遗传发现，最常见于 GPP，其次是 ACH。欧洲和亚洲人种 GPP 患者和 ACH 患者的 IL-36RN 突变基因型不同。IL-36 信号通路是主要机制。代谢性疾病是常见的合并症，两种疾病的关节受累发生率为 20.5%-36.4%。斑块型银屑病在 GPP 中比在 ACH 中更为常见。一般来说，ACH，即使是全身性的，也没有全身炎症，而 GPP 可以有或没有全身炎症。ACH 可以在 GPP 之前、同时或之后发生。然而，GPP 和 ACH 的治疗反应似乎不同。ACH 似乎比 GPP 更难治疗，但 GPP 的严重发作会导致发病率和死亡率。尽管 GPP 和 ACH 具有相同的基因型和发病机制，但我们认为 ACH 应与 GPP 分开分类，而不是作为 GPP 的诊断。未来的研究有必要充分区分这两种情况。
