Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Clin Exp Dermatol. 2020 Aug;45(6):673-678. doi: 10.1111/ced.14234. Epub 2020 May 30.
Morphoea, also known as localized scleroderma, is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Paediatric-onset disease is not uncommon and is associated with frequent relapses. The disease has complex pathogenetic mechanisms and multiple clinical subtypes, and affects children of all ages. Recent research has focused on elucidating the disease pathophysiology and identifying measures of disease activity. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected, and results were analysed before being summarized. In the first of this two-part review, we provide a bird's-eye view of the current literature concerning the epidemiology, aetiopathogenesis and clinical classification of paediatric morphoea; in Part 2, we review the diagnosis, markers of disease activity, management and natural history.
硬斑病，又称局限性硬皮病，是一种病因不明的进行性纤维性疾病，影响皮肤和皮下组织。儿科发病并不少见，且常反复发作。该病发病机制复杂，临床亚型多样，可影响各年龄段儿童。目前的研究重点在于阐明疾病的病理生理学并确定疾病活动的衡量标准。我们在 PubMed、MEDLINE 和 Google Scholar 上使用了“儿科硬斑病”“青少年局限性硬皮病”和“青少年系统性硬化症”等关键词进行文献检索，选择了相关的随机试验、标准现行指南综述和原始研究文章，并在总结之前对结果进行了分析。在这篇两部分综述的第一部分中，我们对儿科硬斑病的流行病学、发病机制和临床分类的现有文献进行了综述；在第二部分中，我们回顾了该病的诊断、疾病活动标志物、治疗和自然病程。
