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硬皮病手部病变:疾病表现与治疗方法

The Scleroderma Hand: Manifestations of Disease and Approach to Management.

作者信息

Williams Ariel A, Carl Hannah M, Lifchez Scott D

机构信息

Department of Plastic and Reconstructive Surgery, Johns Hopkins Medicine, Baltimore, MD.

Johns Hopkins University School of Medicine, Baltimore, MD.

出版信息

J Hand Surg Am. 2018 Jun;43(6):550-557. doi: 10.1016/j.jhsa.2018.03.021.

Abstract

Scleroderma is a rare autoimmune connective tissue disorder that often affects the hands. Manifestations in the hands include calcium deposits within the soft tissues that cause pain and may ulcerate through the skin, digital ischemia resulting in chronic wounds and digital gangrene, and joint contracture. Because of the underlying disease, patients with scleroderma have poorly vascularized tissue and a deficient soft tissue envelope, which make surgery particularly challenging. However, when undertaken with care, surgical intervention is often the best option for addressing the disabling hand conditions that so often accompany this disease.

摘要

硬皮病是一种罕见的自身免疫性结缔组织疾病,常累及手部。手部表现包括软组织内的钙沉积,可引起疼痛并可能穿透皮肤形成溃疡;手指缺血导致慢性伤口和手指坏疽;以及关节挛缩。由于潜在疾病的原因,硬皮病患者的组织血管化不良且软组织包膜不足,这使得手术极具挑战性。然而,如果谨慎进行,手术干预通常是解决这种疾病常伴有的致残性手部状况的最佳选择。

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