红皮病型银屑病:避免住院的卓越管理。

Erythrodermic Psoriasis: Excellent Management Avoiding Hospitalization.

作者信息

Gerosa Silvia, Ravaglia Giorgia, Fantini Carolina, Feliciani Claudio, Satolli Francesca

机构信息

Silvia Gerosa, MD, Section of Dermatology, Department of Medicine and Surgery, University of Parma, Via Gramsci 14, 43126 - Parma, Italy;

出版信息

Acta Dermatovenerol Croat. 2024 Dec;32(4):214-216.

DOI:
Abstract

Erythrodermic psoriasis (EP) is a rare but severe variant of this inflammatory cutaneous disease, occurring in less than 3% of patients with psoriasis. It is characterized by involvement of more than 90% of body surface area (BSA), with diffuse scaling and erythema. Usually, EP develops in subjects with poor control of psoriatic disease (1). EP is considered an emergency condition in dermatology due to extensive skin involvement and systemic symptoms; moreover, it is often resistant to conventional therapies (1,2). While the pathogenesis of plaque psoriasis is well-understood, with a complex interplay between Th1, Th2, and Th17 responses, the inflammatory mechanisms of EP are less known, but the IL-17 pathway seems to play a pivotal role (2). Brodalumab is a fully human monoclonal antibody blocking the interleukin-17 receptor A, thus interfering with different isoforms of IL-17 (A, A/F, F, C, and E) (3). This results in a complete block of the IL-17 response, including IL-17 C and E, which are released by keratinocytes and not directly by the Th17 line. Therefore, brodalumab presents a broader action in comparison with anti-IL-23 and other anti-IL-17 drugs, which act upstream on the Th17 line (4). As shown by Yamasaki et al. in a 52-week open-label study, brodalumab is associated with a rapid response, even in patients with EP, showing a drastic improvement in symptoms after just two weeks (3). Herein we report a case of a 57 -year-old woman with a recent diagnosis of plaque psoriasis, naïve to systemic therapies, who rapidly developed EP. Psoriasis diagnosis was confirmed by skin biopsy. With regard to comorbidities, she presented a history of excessive alcohol use and tested positive for latent tuberculosis. At the first evaluation, the patient presented with BSA involvement of 90%, a PASI score of 42, and a DLQI of 26, without psoriatic arthritis (Figure 1, a). At the examination, a concomitant ocular involvement was particularly evident, with conjunctival redness and a reported burning sensation (Figure 1, b). After receiving prophylactic treatment for latent tuberculosis, brodalumab was initiated at the labeled dosage. A dramatic improvement was observed after just two weeks, with a reduction of erythema and scaliness as well as the itching and burning sensation (Figure 2, a). Furthermore, the conjunctive redness completely disappeared. After 4 weeks, the PASI score was reduced to 2 and BSA decreased to 5%, with a positive impact on quality of life (Figure 2, b). The patient did not report any adverse events. Due to the rarity of this form of psoriasis, international guidelines or recommendations on EP treatment and management are lacking. Several biologic drugs are currently being used off label based on case reports or small case series, with an optimal response and tolerance profile (1). To our knowledge, , there have been only six cases of EP treated with brodalumab in real-life settings (2,4,5). Our experience, in accordance with the cases published in literature, showed a rapid onset of action, without any relevant adverse events. One of the most promising aspects of brodalumab in EP is the reduction in the hospitalization of patients; in fact, thanks to the rapidity of its action it is possible to avoid the administration of systemic steroid therapy, frequently used in the management of EP, and therefore avoid steroid-related AEs. Furthermore, it can be used in monotherapy due to its high efficacy, without conventional immunosuppressive drugs. Finally, its excellent tolerance profile allows its use in a wider patient setting. In conclusion, brodalumab could represent a valid therapeutic option for EP, based on its clinical efficacy, rapid effect, and safety, especially considering the reduction of the clinical burden for both patient and hospital management.

摘要

红皮病型银屑病(EP)是这种炎症性皮肤病的一种罕见但严重的变体,在银屑病患者中发生率不到3%。其特征是体表面积(BSA)超过90%受累,伴有弥漫性鳞屑和红斑。通常,EP发生在银屑病病情控制不佳的患者中(1)。由于广泛的皮肤受累和全身症状,EP在皮肤科被视为一种紧急情况;此外,它通常对传统疗法耐药(1,2)。虽然斑块状银屑病的发病机制已得到充分理解,涉及Th1、Th2和Th17反应之间的复杂相互作用,但EP的炎症机制尚不太清楚,但IL-17途径似乎起着关键作用(2)。布罗达单抗是一种完全人源化单克隆抗体,可阻断白细胞介素-17受体A,从而干扰IL-17的不同异构体(A、A/F、F、C和E)(3)。这导致IL-17反应完全受阻,包括由角质形成细胞而非直接由Th17细胞系释放的IL-17 C和E。因此,与抗IL-23和其他抗IL-17药物相比,布罗达单抗具有更广泛的作用,后者作用于Th17细胞系的上游(4)。正如Yamasaki等人在一项为期52周的开放标签研究中所示,布罗达单抗即使对EP患者也有快速反应,仅两周后症状就有显著改善(3)。在此,我们报告一例57岁女性,近期诊断为斑块状银屑病,未接受过系统治疗,迅速发展为EP。皮肤活检确诊为银屑病。关于合并症,她有过量饮酒史,潜伏性结核检测呈阳性。在首次评估时,患者的BSA受累率为90%,银屑病面积和严重程度指数(PASI)评分为42,皮肤病生活质量指数(DLQI)为26,无银屑病关节炎(图1,a)。检查时,眼部合并受累尤为明显,结膜发红并有烧灼感(图1,b)。在接受潜伏性结核的预防性治疗后,开始按标记剂量使用布罗达单抗。仅两周后就观察到显著改善,红斑、鳞屑减少,瘙痒和烧灼感减轻(图2,a)。此外,结膜发红完全消失。4周后,PASI评分降至2,BSA降至5%,对生活质量有积极影响(图2,b)。患者未报告任何不良事件。由于这种银屑病形式罕见,缺乏关于EP治疗和管理的国际指南或建议。目前,基于病例报告或小病例系列,几种生物药物正在超说明书使用,具有最佳的反应和耐受性(1)。据我们所知,在现实生活中仅有6例EP患者接受了布罗达单抗治疗(2,4,5)。我们的经验与文献中发表的病例一致,显示起效迅速,无任何相关不良事件。布罗达单抗在EP中最有前景的方面之一是减少患者住院;事实上,由于其作用迅速,可以避免使用常用于EP管理的全身类固醇疗法,从而避免与类固醇相关的不良事件。此外,由于其高效性,它可以单药使用,无需传统免疫抑制药物。最后,其出色 的耐受性使其可用于更广泛的患者群体。总之,基于其临床疗效、快速起效和安全性,尤其是考虑到减轻患者和医院管理的临床负担,布罗达单抗可能是EP的一种有效治疗选择。

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