关键词: Biologic agents Eritrodermia Erythroderma Pitiriasis rubra pilaris Pityriasis rubra pilaris Tratamiento biológico

Mesh : Humans Male Pityriasis Rubra Pilaris / pathology drug therapy Retrospective Studies Female Middle Aged Spain / epidemiology Aged Adult Child Adolescent Child, Preschool Young Adult Aged, 80 and over Dermatitis, Exfoliative / etiology pathology Treatment Outcome Adrenal Cortex Hormones / therapeutic use Infant

来  源:   DOI:10.1016/j.ad.2024.02.019

Abstract:
BACKGROUND: PRP is a rare entity of unknown etiopathogenesis. Lack of management guidelines makes it a challenge for clinicians.
OBJECTIVE: To add our experience to increase evidence about PRP.
METHODS: We performed a retrospective, descriptive and multicentric study of 65 patients with PRP, being the largest European case series of patients with PRP.
RESULTS: PRP was more frequent in male patients with an average age of 51 years, but erythrodermic forms presented in older patients (average age 61 years). Six (75%) paediatric patients and ten (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. On the contrary, 26 (68%) erythrodermic patients required biologic therapy as last and effective therapy line requiring an average of 6.5 months to achieve complete response.
CONCLUSIONS: Our study showed a statistical difference in terms of outcome and response to treatment between children or patients with limited disease and patients who develop erythroderma.
摘要:
背景:PRP是一种未知病因的罕见实体。缺乏临床实践指南使临床医生的管理具有挑战性。
目的:将我们的经验添加到PRP的语料库证据中。
方法:这是一个回顾性研究,描述性,描述性65例PRP患者的多中心研究,欧洲有史以来最大的PRP患者病例系列报告.
结果:PRP在平均年龄为51岁的男性中更为普遍,然而,在老年患者中出现红皮病型(平均年龄,61年)。6名(75%)儿科患者和10名(60%)非红皮病患者成年人通过局部皮质类固醇控制了疾病。然而,26例(68%)红皮病患者需要生物疗法作为最后的有效疗法,平均6.5个月才能达到完全缓解。
结论:我们的研究表明,儿童在治疗结果和反应方面存在统计学差异,或患有局限性疾病的患者和患有红皮病的患者。
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