From New York Hand & Wrist Center of Lenox Hill (Dr. Beldner and Dr. Polatsch), and the Department of Orthopaedic Surgery, Lenox Hill Hospital (Dr. Rabinovich), New York, NY.
J Am Acad Orthop Surg. 2020 Aug 15;28(16):e686-e695. doi: 10.5435/JAAOS-D-19-00547.
Scleroderma is derived from Latin meaning hard skin. It is an uncommon, noninflammatory connective tissue disorder characterized by increased fibrosis of the skin and in certain variants, multiple other organ systems. Scleroderma involves a spectrum of pathologic changes and anatomic involvement. It can be divided into localized and systemic scleroderma. Hand involvement is common and can include calcium deposits within the soft tissues, digital ischemia, and joint contracture. Nonsurgical management consists of lifestyle modifications, biofeedback, therapy for digital stiffness/contracture, and various pharmacologic medications. When nonsurgical measures are unsuccessful, certain surgical options may be indicated, each with their inherent advantages and pitfalls. Patients with scleroderma who are undergoing surgical intervention pose unique difficulties because of their poorly vascularized tissue and deficient soft-tissue envelopes, thus increasing their susceptibility to wound healing complications and infection. Some subgroups of patients are frequently systemically ill, and specific perioperative measures should be considered to reduce their surgical risk. The spectrum of hand manifestations seen in patients with scleroderma will be reviewed with the focus on evaluation and management.
硬皮病源自拉丁语，意为“硬皮”。它是一种罕见的非炎症性结缔组织疾病，其特征是皮肤纤维化增加，在某些变体中，还会影响多个其他器官系统。硬皮病涉及一系列病理变化和解剖学受累。它可以分为局限性和系统性硬皮病。手部受累很常见，可能包括软组织内钙沉积、手指缺血和关节挛缩。非手术治疗包括生活方式改变、生物反馈、治疗手指僵硬/挛缩以及各种药物治疗。当非手术治疗无效时，可能需要某些手术选择，每种选择都有其固有的优点和缺点。由于组织血供不良和软组织缺乏，接受手术干预的硬皮病患者会出现独特的困难，从而增加了他们发生伤口愈合并发症和感染的风险。某些亚组的患者经常患有系统性疾病，因此应考虑特定的围手术期措施来降低手术风险。本文将回顾硬皮病患者手部表现的范围，并重点介绍评估和治疗。
