Mastorino Luca, Leo Francesco, Frigatti Giada, Macagno Nicole, Dapavo Paolo, Quaglino Pietro, Ribero Simone
Section of Dermatology, Department of Medical Sciences, University of Turin, Via Cherasco 23, 10126, Turin, Italy.
Am J Clin Dermatol. 2025 Aug 26. doi: 10.1007/s40257-025-00977-1.
Erythrodermic psoriasis (EP) is a severe and rare variant of psoriasis. Clinical features include scaling and erythema affecting more than 75% of body surface area, associated with systemic symptoms such as lymphadenopathy, arthralgia, fever, fatigue, dehydration, serum electrolyte disturbances, and tachycardia, making this condition a potentially life-threatening disease. Differential diagnosis can be challenging, encompasses atopic dermatitis, cutaneous adverse drug reaction, and advanced cutaneous lymphoma. Following a correct diagnostic framing, appropriate systemic treatment must be initiated. Unfortunately, there are no recent up-to-date guidelines and standardized treatment options for EP are still lacking.
To review the current reported systemic treatment options for EP.
This systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, and based on a search in MEDLINE, PubMed, Scopus, and Cochrane Library for articles in English from first available publication to 9 November 2024.
In all, 145 studies were included in the review. Case reports and case series are the main available work, reporting heterogeneous outcomes and effectiveness with nonbiologic and biologic systemic agents. Among non-biologic systemic treatments, methotrexate and cyclosporin are the most widely reported as treatment for EP, showing clinical response in over 60% of cases, with cyclosporine offering a faster onset of action and being suitable for acute management. Available randomized controlled trials include patients with EP treated with etretinate, infliximab, certolizumab-pegol (CZP), Ixekizumab, guselkumab, risankizumab, and deucravacitinib. However, these trials were not specifically designed for erythrodermic psoriasis, and the sample size of EP patients included is limited, resulting in reduced statistical power and limiting the reliability of the findings. Among TNF-α inhibitors, infliximab is the most reported agent, with data on 103 patients. Certolizumab pegol (CZP) also showed promising results, with PASI 75 achieved in over 80% of patients at 52 weeks. A retrospective analysis comparing infliximab, adalimumab, etanercept, ustekinumab, and efalizumab found TNF-α inhibitors to be superior to other biologic classes. Regarding IL-17 inhibitors, secukinumab is the second most frequently studied biologic, with 93 patients reported. It demonstrated rapid efficacy, achieving PASI 75 in more than 80% of patients by week 8. A head-to-head comparison with ixekizumab showed comparable outcomes. Among IL-23 inhibitors, risankizumab led to PASI 90 in over 75% of patients at week 16, suggesting high efficacy despite more limited data.
Non-biologic systemic drugs appear to be a rational first-line therapy, with cyclosporine showing good results in managing the acute phase and methotrexate being effective in maintaining remission. In the case of contraindications or treatment failure of traditional systemic therapies, among biologic drugs, the rapidity of action, safety, and limited evidence of efficacy are in favor of IL-17 inhibitors and risankizumab. However, the findings we report are limited by the evidence available in current literature, which is characterized by low statistical power.
红皮病型银屑病（EP）是银屑病的一种严重且罕见的类型。临床特征包括鳞屑和红斑累及超过75%的体表面积，并伴有全身症状，如淋巴结病、关节痛、发热、疲劳、脱水、血清电解质紊乱和心动过速，使这种疾病成为一种潜在的危及生命的疾病。鉴别诊断具有挑战性，包括特应性皮炎、皮肤药物不良反应和晚期皮肤淋巴瘤。在进行正确的诊断框架构建后，必须启动适当的全身治疗。不幸的是，目前尚无最新的指南，且仍缺乏针对EP的标准化治疗方案。
综述目前报道的EP全身治疗方案。
本系统评价按照系统评价和Meta分析的首选报告项目（PRISMA）指南进行，基于对MEDLINE、PubMed、Scopus和Cochrane图书馆中从首次发表至2024年11月9日的英文文章的检索。
本评价共纳入145项研究。病例报告和病例系列是主要的现有研究，报告了使用非生物和生物全身药物的异质性结果和有效性。在非生物全身治疗中，甲氨蝶呤和环孢素作为EP治疗的报道最为广泛，超过60%的病例显示出临床反应，环孢素起效更快，适用于急性处理。现有的随机对照试验包括接受阿维A、英夫利昔单抗、赛妥珠单抗（CZP）、依奇珠单抗、古塞库单抗、司库奇尤单抗和氘可来昔替尼治疗的EP患者。然而，这些试验并非专门针对红皮病型银屑病设计，纳入的EP患者样本量有限，导致统计效能降低，限制了研究结果的可靠性。在肿瘤坏死因子-α（TNF-α）抑制剂中，英夫利昔单抗是报道最多的药物，有103例患者的数据。赛妥珠单抗（CZP）也显示出有前景的结果，52周时超过80%的患者达到银屑病面积和严重程度指数（PASI）改善75%。一项比较英夫利昔单抗、阿达木单抗、依那西普、乌司奴单抗和依法利珠单抗的回顾性分析发现，TNF-α抑制剂优于其他生物制剂类别。关于白细胞介素-17（IL-17）抑制剂，司库奇尤单抗是研究第二频繁的生物制剂，有93例患者的报道。它显示出快速疗效，第8周时超过80%的患者达到PASI改善75%。与依奇珠单抗的头对头比较显示出相似的结果。在白细胞介素-23（IL-23）抑制剂中，司库奇尤单抗在第16周时超过75%的患者达到PASI改善90%，尽管数据有限，但表明疗效较高。
非生物全身药物似乎是合理的一线治疗方法，环孢素在急性期处理中显示出良好效果，甲氨蝶呤在维持缓解方面有效。在传统全身治疗存在禁忌证或治疗失败的情况下，在生物药物中，IL-17抑制剂和司库奇尤单抗在作用速度、安全性和有限的疗效证据方面具有优势。然而，我们报告的研究结果受到当前文献可用证据的限制，其特点是统计效能较低。
