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局限性硬皮病:影像学特征

Localized scleroderma: imaging features.

作者信息

Liu P, Uziel Y, Chuang S, Silverman E, Krafchik B, Laxer R

机构信息

Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada.

出版信息

Pediatr Radiol. 1994;24(3):207-9. doi: 10.1007/BF02012193.

DOI:10.1007/BF02012193
PMID:7936801
Abstract

Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with "en coup de sabre" facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients.

摘要

局限性硬皮病与弥漫性硬皮病不同,不表现雷诺现象和内脏受累。回顾了23例年龄在2至17岁(平均11.1岁)患者的影像学特征。下肢长度差异和肌肉萎缩是最常见的表现(5例患者),2例患者还出现腓骨塑形畸形。1例下肢受累患者的磁共振成像显示骨髓信号异常。1例患者出现需要骨科干预的致残性关节挛缩。2例“剑伤样”面部畸形患者的CT和磁共振成像扫描显示同侧额叶颅内钙化和白质异常,其中1例还显示移行异常。第3例患者的CT显示同侧顶叶白质异常。1例进行性面部半侧萎缩患者的CT和磁共振成像扫描显示左侧上颌窦和脸颊发育不全。对临床关注区域的影像学研究发现,半数患者有阳性结果。

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2
Progressive facial hemiatrophy: central nervous system involvement and relationship with scleroderma en coup de sabre.进行性面部半侧萎缩:中枢神经系统受累及与剑伤样硬皮病的关系。
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Fracture risk in linear morphea-affected limbs: A pilot retrospective study of adult patients.线状硬皮病累及肢体的骨折风险:一项针对成年患者的回顾性试点研究。
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进行性系统性硬化症(硬皮病)的骨骼表现
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