Boin Francesco, Hummers Laura K
Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Mason F. Lord Building, Center Tower, Suite 4100, Room 405, Baltimore, MD 21224, USA.
Rheum Dis Clin North Am. 2008 Feb;34(1):199-220; ix. doi: 10.1016/j.rdc.2007.11.001.
Many conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology, and disease associations may allow one to distinguish these conditions from scleroderma and from each other. A prompt diagnosis is important to spare patients from ineffective treatments and inadequate management. This article highlights nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy), eosinophilic fasciitis (Shulman's syndrome), scleromyxedema, and scleredema. These often are detected in the primary care setting and referred to rheumatologists for further evaluation. Rheumatologists must be able to promptly recognize them to provide valuable prognostic information and appropriate treatment options for affected patients.
许多表现为临床硬皮和组织纤维化的病症可能会与系统性硬化症（硬皮病）相混淆。这些疾病病因极为多样，发病机制往往也不明确。不同的临床特征、皮肤组织学表现及疾病关联可能有助于将这些病症与硬皮病区分开来，也有助于相互鉴别。及时诊断对于避免患者接受无效治疗和管理不当至关重要。本文重点介绍了肾源性系统性纤维化（肾源性纤维化皮肤病）、嗜酸性筋膜炎（舒尔曼综合征）、硬化性黏液水肿和硬化性水肿。这些病症常在初级保健机构中被发现，并转诊给风湿病学家做进一步评估。风湿病学家必须能够迅速识别它们，以便为受影响的患者提供有价值的预后信息和合适的治疗方案。
