Recalcitrant pityriasis rubra pilaris in a Middle Eastern patient and arguments for early anti-IL-23 targeting

Mohammed N. Al-Abdulla, Wadha Al-Shafi, Hanof Ahmed, Anh Jochebeth, Febu Joy, Shahd Younis, Mahir Petkar, Joerg Buddenkotte, Martin Steinhoff
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Abstract

Pityriasis rubra pilaris (PRP) is a rare, chronic cutaneous inflammatory disorder of keratinization affecting adults, children and patients with HIV. The pathogenesis of PRP is not fully understood with clinical presentations, and severity remains highly variable. Current treatment modalities for PRP result in recalcitrant disease with potentially unfavourable therapeutic side effects and low tolerability. Due to the rarity of this condition, limited data on treatment efficacies and established management guidelines are lacking. The psychological burden of PRP is detrimental to the quality of life of patients affected with PRP and remains a persistent gap of knowledge. Here, we provide a review of the literature, summarizing new developments in the treatment of PRP and a case report of a patient treated successfully with the anti-interleukin (IL)-23p19 antibody Risankizumab with sustained clinical improvement. Risankizumab appears to be an effective and safe treatment for PRP in Asian-Arabic patients. Further studies are required to assess the efficacy, safety and tolerability of newer targeted therapies for severe PRP.

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一名中东患者顽固的红斑狼疮和早期抗IL-23靶向治疗的论据
红斑性瘙痒症(PRP)是一种罕见的慢性角化性皮肤炎症,成人、儿童和艾滋病患者均可患病。PRP 的发病机制尚不完全清楚,临床表现和严重程度也千差万别。目前针对 PRP 的治疗方法会导致疾病反复发作,并可能带来不利的治疗副作用和较低的耐受性。由于这种疾病的罕见性,有关治疗效果和既定管理指南的数据十分有限。PRP 带来的心理负担不利于 PRP 患者的生活质量,这仍然是一个长期的知识空白。在此,我们对文献进行了综述,总结了 PRP 治疗的新进展,并报告了一例使用抗白细胞介素(IL)-23p19 抗体 Risankizumab 成功治疗并持续改善临床症状的病例。利桑珠单抗似乎是治疗亚洲-阿拉伯患者 PRP 的一种有效而安全的方法。还需要进一步的研究来评估新的靶向疗法对严重 PRP 的疗效、安全性和耐受性。
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