幼年局部硬皮病的挑战与并发症：实用方法。

IF 4.5 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology Pub Date : 2024-09-01 DOI:10.1016/j.berh.2024.101987

Clare E. Pain , Kathryn S. Torok

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引用次数: 0

摘要

青少年局部硬皮病的特点是炎症导致皮肤和软组织纤维化。局部硬皮病中较严重的亚型，如线性硬皮病和颅面硬皮病，在儿童中更为常见。此外，半数患儿会出现皮肤外受累，这与较差的治疗效果和与健康相关的生活质量有关。颅面和皮外受累的治疗缺乏证据，因此给临床医生带来了挑战。本综述旨在通过病例研究，介绍现有证据、当前建议和管理注意事项，为管理这些最具挑战性的幼年局部硬皮病特征提供实用方法。

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Challenges and complications in juvenile localized scleroderma: A practical approach

Juvenile localized scleroderma is characterised by inflammation which drives fibrosis in skin and soft tissues. The more severe subtypes of localized scleroderma such as linear and craniofacial are more common in children. Additionally, extracutaneous involvement is seen in half of all children and is associated with poorer treatment outcomes and health-related quality of life. Evidence for the management of craniofacial and extracutaneous involvement is lacking and therefore poses a challenge to clinicians. This review aims to provide a practical approach to management of these most challenging features of juvenile localized scleroderma through case studies where we present the available evidence, current recommendations and considerations for management.

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来源期刊

Best Practice & Research in Clinical Rheumatology 医学-风湿病学

CiteScore

9.40

自引率

0.00%

发文量

审稿时长

27 days

期刊介绍： Evidence-based updates of best clinical practice across the spectrum of musculoskeletal conditions. Best Practice & Research: Clinical Rheumatology keeps the clinician or trainee informed of the latest developments and current recommended practice in the rapidly advancing fields of musculoskeletal conditions and science. The series provides a continuous update of current clinical practice. It is a topical serial publication that covers the spectrum of musculoskeletal conditions in a 4-year cycle. Each topic-based issue contains around 200 pages of practical, evidence-based review articles, which integrate the results from the latest original research with current clinical practice and thinking to provide a continuous update. Each issue follows a problem-orientated approach that focuses on the key questions to be addressed, clearly defining what is known and not known. The review articles seek to address the clinical issues of diagnosis, treatment and patient management. Management is described in practical terms so that it can be applied to the individual patient. The serial is aimed at the physician in both practice and training.