Clinical Epidemiology Unit, Istituto Dermopatico dell'Immacolata (IDI)-IRCCS-FLMM, Rome, Italy.
I Dermatology Division, Istituto Dermopatico dell'Immacolata (IDI)-IRCCS-FLMM, Rome, Italy.
J Dermatol. 2018 Feb;45(2):202-206. doi: 10.1111/1346-8138.14114. Epub 2017 Oct 28.
Pityriasis rubra pilaris (PRP) is a rare, chronic, inflammatory skin disease of unknown etiology. Patients refractory to conventional therapies have been treated successfully with biologic drugs such as anti-tumor necrosis factor agents. Recently, a role of the interleukin-23/T-helper 17 axis in PRP has been described. Our objective was to assess the effectiveness of ustekinumab in five patients with adult-onset PRP refractory to conventional therapies. In the present study, four patients had type I and one patient type II adult-onset PRP. They were treated with three s.c. doses of ustekinumab at weeks 0, 4 and 16. Clinical response was evaluated monthly during treatment up to a 15-month follow-up period. All patients promptly showed a decrease in erythema, follicular hyperkeratosis and scaling. After three injections, complete remission of skin lesions was achieved in four out of five cases and a significant clinical improvement was shown in one case. To the best of our knowledge, this is the largest case series reported on ustekinumab treatment in PRP. Our results, in addition to previous studies from other groups, suggest that ustekinumab may be a possible first-line treatment for PRP patients refractory to conventional therapies.
红糠疹（PRP）是一种罕见的、慢性的、病因不明的炎症性皮肤病。对于常规治疗方法无效的患者，生物药物如抗肿瘤坏死因子制剂已成功用于治疗。最近，人们描述了白细胞介素-23/T 辅助 17 轴在 PRP 中的作用。我们的目的是评估乌司奴单抗在五例对常规治疗方法难治的成人发病型 PRP 患者中的疗效。在本研究中，四例患者为 I 型，一例患者为 II 型成人发病型 PRP。他们在第 0、4 和 16 周接受了三次皮下注射乌司奴单抗。在治疗期间，每月评估一次临床反应，随访时间长达 15 个月。所有患者的红斑、毛囊角化过度和鳞屑迅速减少。在三次注射后，五例患者中有四例皮损完全缓解，一例患者的临床症状显著改善。据我们所知，这是乌司奴单抗治疗 PRP 的最大病例系列报道。我们的结果以及其他组的先前研究表明，乌司奴单抗可能是对常规治疗方法无效的 PRP 患者的一种可能的一线治疗药物。
