Abstract：
Cutaneous Rosai Dorfman disease (CRDD) is a rare histiocytic disorder that shows distinctive clinical presentation and prognosis. Sufficient data is currently lacking regarding evidence-based management of CRDD. This systematic review aims to provide a comprehensive overview of CRDD, focusing on treatment approaches and outcomes. PubMed and Scopus databases were searched for studies on CRDD from June 1st, 2013 to May 31st, 2023. Articles describing cases of CRDD confirmed with histological examination were eligible for inclusion. All interventions for CRDD were analyzed. The primary outcome measure was the response of cutaneous lesions to treatment including complete response (CR), partial response (PR), and no response. The secondary outcome measures were mortality rate, relapse rate, and the occurrence of adverse events related to CRDD treatment. Eighty-seven articles describing 118 CRDD cases were included. The mean age was 48.2±16.8 years. The sex ratio (F/M) was 1.53. Nodular (46.6%) erythematous (45.3%) lesions, located on the face (38.1%) were the most prevalent presentations. Associated hematological malignancies were noted in 8 (6.8%) cases. Surgical excision was the most prevalent intervention (51 cases) with CR in 48 cases. Systemic corticosteroids were used in 32 cases with 20 CR/PR, retinoids in 10 cases with 4 CR/PR, thalidomide in 9 cases with 5 CR/PR, methotrexate in 8 cases with 7 CR/PR while observation was decided in 10 cases with 6 CR/PR. Factors independently associated with the absence of response to treatment were facial involvement (OR = 0.76, p = 0.014), and cutaneous lesion size (OR = 1.016, p = 0.03). This systematic review shows distinctive clinical characteristics of CRDD and provides insights into the appropriate management of the disease. It allowed a proposal of a treatment algorithm that should be interpreted in the context of current evidence and would help practitioners in treating this rare disease.
摘要：
皮肤RosaiDorfman病（CRDD）是一种罕见的组织细胞疾病，表现出独特的临床表现和预后。目前缺乏关于CRDD循证管理的足够数据。本系统综述旨在全面概述CRDD，关注治疗方法和结果。从6月1日起，搜索PubMed和Scopus数据库以进行CRDD研究，2013年5月31日，2023年。描述经组织学检查证实的CRDD病例的文章符合纳入条件。对CRDD的所有干预措施进行分析。主要结果指标是皮肤病变对治疗的反应，包括完全缓解（CR），部分响应（PR），也没有回应.次要结果指标是死亡率，复发率,以及与CRDD治疗相关的不良事件的发生。纳入了87篇描述118例CRDD病例的文章。平均年龄为48.2±16.8岁。性别比例(F/M)为1.53。结节性（46.6%）红斑（45.3%）病变，位于面部（38.1％）是最普遍的表现。在8例（6.8％）中发现了相关的血液恶性肿瘤。手术切除是最普遍的干预措施（51例），其中48例CR。32例采用全身糖皮质激素治疗，CR/PR20例，10例CR/PR为4例，5CR/PR的沙利度胺9例，甲氨蝶呤8例CR/PR7例，观察10例CR/PR6例。与治疗无反应独立相关的因素是面部受累（OR=0.76，p=0.014），和皮肤病变大小（OR=1.016，p=0.03）。该系统综述显示了CRDD的独特临床特征，并提供了对该疾病的适当管理的见解。它允许提出一种治疗算法，该算法应在当前证据的背景下进行解释，并将帮助从业者治疗这种罕见疾病。
