Department of Dermatology, Policlinico Agostino Gemelli, Catholic University Sacred Heart, Rome, Italy.
J Eur Acad Dermatol Venereol. 2010 Aug;24(8):881-4. doi: 10.1111/j.1468-3083.2009.03511.x. Epub 2009 Nov 30.
Pityriasis rubra pilaris (PRP) is a rare inflammatory dermatosis with frequent clinical presentation as erythroderma. Conventional systemic treatment is often unsatisfactory and limited by long-term toxicity. The use of tumour necrosis factor (TNF) antagonists has been reported previously in single cases, but lacking long-term follow-up or comparison between different biological agents.
To assess the long-term efficacy and safety of TNF-alpha antagonist, infliximab and etanercept, either in monotherapy or in combination therapy of severe, refractory adult-onset PRP.
Seven patients of adult-onset PRP, six newly diagnosed type-I and 1 type-II, which were resistant or ineligible to conventional systemic treatment, received a single course of infliximab or etanercept therapy, alone or in combination with low-dose acitretin (>0.25 mg/kg/daily). After complete remission and treatment discontinuation, a follow-up period of 12 months was evaluated for relapses.
Six patients obtained complete remission after a single course of anti-TNF-alpha therapy: mean therapy duration was 19.3 weeks (range 6-48 weeks). All patients obtained significant clearing (>75% of body surface area) of skin lesions at week 12. Two patients with marked keratoderma developed localized disease recurrence during treatment. During follow-up, only a single patient, affected by type II PRP, had disease relapse.
Both TNF-alpha antagonists proved successful for the treatment of refractory, adult-onset PRP, yielding complete and persistent clinical responses in type-I PRP. Infliximab was associated with a more rapid onset of action, while treatment duration was comparable with etanercept. PRP type II warranted long-term therapy and showed relapse after drug discontinuation.
毛发红糠疹（PRP）是一种罕见的炎症性皮肤病，常表现为红皮病。传统的全身治疗往往效果不佳，并受到长期毒性的限制。先前已有报道使用肿瘤坏死因子（TNF）拮抗剂治疗单例病例，但缺乏长期随访或不同生物制剂之间的比较。
评估 TNF-α拮抗剂英夫利昔单抗和依那西普在单药或联合治疗成人发病的严重、难治性 PRP 中的长期疗效和安全性。
7 例成人发病的 PRP 患者，6 例新诊断为 I 型，1 例为 II 型，对常规全身治疗耐药或不适合，接受了英夫利昔单抗或依那西普单一疗程治疗，单独或联合低剂量阿维 A（>0.25mg/kg/天）。完全缓解并停药后，评估了 12 个月的复发随访期。
6 例患者在接受单次抗 TNF-α治疗后获得完全缓解：平均治疗持续时间为 19.3 周（范围 6-48 周）。所有患者在第 12 周时皮肤病变明显清除（>75%的体表面积）。2 例有明显角化过度的患者在治疗期间出现局部疾病复发。在随访期间，仅 1 例 II 型 PRP 患者出现疾病复发。
两种 TNF-α拮抗剂均成功治疗难治性成人发病 PRP，在 I 型 PRP 中产生完全和持久的临床反应。英夫利昔单抗起效更快，而治疗持续时间与依那西普相当。PRP Ⅱ型需要长期治疗，并在停药后出现复发。
