局限性硬皮病中国皮肤科诊疗专家共识

标题: 局限性硬皮病中国皮肤科诊疗专家共识
title: Chinese expert concensus on the diagnosis and treatment of localized scleroderma
版本: 原创版
version: Original
分类: 专家共识
classification: Experts consensus
领域: 诊疗
field: Diagnosis and Treatment
国家和地区: 中国
Country and region: China
指南使用者: 皮肤科医生
Guide users: Dermatologist
证据分级方法: GRADE证据质量分级和推荐强度评价系统进行证据评价,并参照指南共识研究和评价工具(STAR)标准和要求[8),结合临床实践制订。
Evidence grading method: GRADE method
制定单位: 中南大学湘雅二医院,中国医学科学院皮肤病研究所,中国医科大学附属第一医院
Formulating unit: The Second Xiangya Hospital of Central South University;Chinese Academy of Medical Sciences and Peking Union Medical College;The First Hospital of China Medical University
注册时间: 2024-10-14
Registration time:
注册编号: PREPARE-2024CN815
Registration number:
指南制订的目的: 局限性硬皮病一种慢性损容性皮肤病,不仅累及皮肤,还可以累及皮下组织、肌肉和骨骼。初期仅表现为皮肤红斑水肿、光泽度改变或轻度色素异常,随疾病进展皮损部位逐渐发生硬化、萎缩,严重时瘢痕形成、毛发缺失等,严重损害患者心理健康和生活质量。一直以来国内对该疾病关注度偏低,尤其是广大基层皮肤科医生对其缺乏全面深入的了解,诊治行为不够规范,部分患者存在漏诊误诊,严重影响患者诊疗进程及预后,对患者及其家庭造成了严重负担,也对皮肤科医生在临床工作能够正确诊断、合理治疗提出了巨大挑战。因此有必要根据我国国情,制定专家共识,以普及、规范诊疗行为。 目前国际上仅欧洲及日本提出了局限性硬皮病相关诊疗指南,其中就疾病的临床表现、辅助检查、治疗方案、预后等方面进行了阐述。而我国尚无针对该疾病的专家共识或指南。 因此,为了进一步的临床应用,本指南拟从局限性硬皮病的临床分类、临床表现、辅助检查、治疗方案等方面进行证据推荐,为临床医生提供指导意见,希望规范行业行为,造福患者。
Purpose of the guideline: Localized scleroderma is a chronic disfiguring skin disease that not only involves the skin but also can involve subcutaneous tissue, muscles and bones. In the initial stage, it is only manifested as skin erythema and edema, changes in glossiness, or mild pigment abnormalities. As the disease progresses, the affected skin area gradually undergoes hardening and atrophy. In severe cases, scar formation and hair loss occur, which seriously damages the mental health and quality of life of patients. For a long time, the domestic attention to this disease has been relatively low. In particular, the majority of grass-roots dermatologists lack a comprehensive and in-depth understanding of it, and the diagnosis and treatment behaviors are not standardized enough. Some patients are misdiagnosed or missed, which seriously affects the diagnosis and treatment process and prognosis of patients, causing a serious burden on patients and their families. It also poses a huge challenge for dermatologists to correctly diagnose and reasonably treat in clinical work. Therefore, it is necessary to formulate expert consensus according to China's national conditions to popularize and standardize diagnosis and treatment behaviors. At present, only Europe and Japan have proposed relevant diagnosis and treatment guidelines for localized scleroderma, which elaborates on aspects such as the disease's clinical manifestations, auxiliary examinations, treatment plans, and prognosis. However, there is currently no expert consensus or guideline for this disease in China. Therefore, for further clinical application, this guideline intends to provide evidence recommendations on aspects such as the clinical classification, clinical manifestations, auxiliary examinations, and treatment plans of localized scleroderma to provide guidance for clinicians, hoping to standardize industry behaviors and benefit patients.