Zulian Francesco
Division of Paediatric Rheumatology, Department of Paediatrics, University of Padova, Padova, Italy.
Curr Opin Rheumatol. 2008 Sep;20(5):601-7. doi: 10.1097/BOR.0b013e328309a5eb.
Localized scleroderma, also known as morphea, is the most frequent form of scleroderma in childhood. Early diagnosis, appropriate assessment and effective treatment may improve the long-term outcome.
Recent studies, regarding the microchimerism theory or the mechanism of action of phototherapy, have yielded important information on the disease etiopathogenesis. Others have added interesting contributions on new outcome measures for the disease assessment and for the development of future therapeutic trials. Previous results, using methotrexate and phototherapy, have been confirmed. A successful use of bosentan, an endothelin receptor antagonist with vasodilatative and antifibrotic properties for refractory cutaneous ulcerations in pansclerotic morphea, opens new horizons of treatment.
Studies over the past year highlight the role of some outcome measures in the disease assessment and monitoring, with important implications both for the clinical practice and future clinical trials.
局限性硬皮病，也称为硬斑病，是儿童期最常见的硬皮病形式。早期诊断、恰当评估和有效治疗可能改善长期预后。
近期关于微嵌合理论或光疗作用机制的研究，已就该疾病的发病机制产生了重要信息。其他研究则为疾病评估和未来治疗试验的开展提供了关于新结局指标的有趣见解。先前使用甲氨蝶呤和光疗的结果已得到证实。波生坦是一种具有血管舒张和抗纤维化特性的内皮素受体拮抗剂，成功用于治疗泛发性硬斑病的难治性皮肤溃疡，为治疗开辟了新视野。
过去一年的研究突出了一些结局指标在疾病评估和监测中的作用，对临床实践和未来临床试验均具有重要意义。
