红斑狼疮:临床表现、发病机制和治疗方案的最新回顾

IF 8.6 1区 医学 Q1 DERMATOLOGY American Journal of Clinical Dermatology Pub Date : 2023-12-30 DOI:10.1007/s40257-023-00836-x
Tejas P. Joshi, Madeleine Duvic
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引用次数: 0

摘要

真菌性红斑狼疮(PRP)是一种罕见的丘疹鳞屑反应模式,对生活质量有很大影响。PRP I 型是最常见的 PRP 变异型,表现为毛囊性红斑丘疹,随后凝聚成斑块,并伴有特征性的岛状疏松;组织学上,正角化和副角化交替出现的模式被认为是 PRP 的特征(棋盘格状角化过度)。其他 PRP 变体(II-V 型)的发病年龄和临床表现各不相同。VI 型 PRP 是一种罕见的 PRP 亚型,与人类免疫缺陷病毒感染有关,偶尔也与毛囊闭塞四联症有关。Caspase 募集结构域家族成员 14(CARD14)相关丘疹鳞屑疹和面部盘状皮炎是新近描述的疾病状态,与 PRP 有重要的临床重叠,在诊断和治疗方面造成了共同的难题。引起 PRP 的病因往往仍不确定;有人认为 PRP 与感染、恶性肿瘤或某些病例中的药物/疫苗注射有关,但这些都是基于病例报告,其因果关系尚未确定。V 型 PRP 通常是由于先天性 CARD14 基因突变所致。此外,最近的文献发现白细胞介素-23/T-helper-17 细胞轴失调是 PRP 发病机制的主要介质,这为机制导向治疗铺平了道路。目前,大剂量异维A酸、ixekizumab和secukinumab是得到单臂前瞻性研究支持的系统性药物;许多其他药物也被试用于PRP的治疗,但成功率不一。在此,我们将讨论临床表现的最新情况,介绍对发病机制的新见解,并对最近描述的治疗方案进行调查。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Pityriasis Rubra Pilaris: An Updated Review of Clinical Presentation, Etiopathogenesis, and Treatment Options

Pityriasis rubra pilaris (PRP) is a rare papulosquamous reaction pattern with a significant impact on quality of life. Type I PRP is the most common PRP variant, presenting as erythematous papules emerging in a follicular distribution and later coalescing into plaques with characteristic islands of sparing; histologically, an alternating pattern of orthokeratosis and parakeratosis is considered the hallmark of PRP (checkerboard hyperkeratosis). Other PRP variants (types II–V) differ in their age of onset and clinical presentation. Type VI PRP is a rare PRP subtype associated with human immunodeficiency virus infection and is occasionally associated with diseases of the follicular occlusion tetrad. Caspase recruitment domain family, member 14 (CARD14)-associated papulosquamous eruption and facial discoid dermatitis are newly described disease states that have an important clinical overlap with PRP, creating shared conundrums with respect to diagnosis and treatment. The etiology inciting PRP often remains uncertain; PRP has been suggested to be associated with infection, malignancy, or drug/vaccine administration in some cases, although these are based on case reports and causality has not been established. Type V PRP is often due to inborn CARD14 mutations. Furthermore, recent literature has identified interleukin-23/T-helper-17 cell axis dysregulation to be a major mediator of PRP pathogenesis, paving the way for mechanism-directed therapy. At present, high-dose isotretinoin, ixekizumab, and secukinumab are systemic agents supported by single-arm prospective studies; numerous other agents have also been trialed for PRP, with variable success rates. Here, we discuss updates on clinical manifestations, present new insights into etiopathogenesis, and offer a survey of recently described therapeutic options.

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来源期刊
CiteScore
15.20
自引率
2.70%
发文量
84
审稿时长
>12 weeks
期刊介绍: The American Journal of Clinical Dermatology is dedicated to evidence-based therapy and effective patient management in dermatology. It publishes critical review articles and clinically focused original research covering comprehensive aspects of dermatological conditions. The journal enhances visibility and educational value through features like Key Points summaries, plain language summaries, and various digital elements, ensuring accessibility and depth for a diverse readership.
期刊最新文献
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