Groove Sign of Eosinophilic Fasciitis.

Groove Sign of Eosinophilic Fasciitis.
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嗜酸性筋膜炎的沟征。

DOI:
10.1097/rhu.0000000000000524
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发表时间:
2017
期刊:
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases
影响因子:
--
通讯作者:
A. Femia
A. Femia
中科院分区:
--
文献类型:
--
作者:
R. Fruchter;D. Mazori;A. Femia

文献摘要

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一位59岁女性,因7个月左右的水肿和进行性双侧小腿和前臂硬结,伴关节挛缩导致的功能受限而就诊。在就诊前,这些发现提示对充血性心力衰竭、水肿和寄生虫感染的推定诊断进行治疗,但均无改善。体格检查显示四肢水肿和硬结,远端手指无损伤。没有相关的雷诺现象或甲襞毛细血管变化。在前臂(图,A)和腿部(图,B),沿着静脉走向的线性凹陷沿着静脉,在肢体抬高时更好地观察到,与沟征一致。外周血嗜酸性粒细胞计数为1980个细胞/μL。磁共振成像显示四肢筋膜增强,这些结果诊断嗜酸性筋膜炎(EF),一种罕见的硬化性筋膜疾病,导致红斑,水肿和四肢硬结。由于关节挛缩导致的功能限制经常发生。由于其罕见性,EF通常在最初被误诊,延误治疗并恶化结果。1、2凹槽标志的识别,这是特征
A59-year-old woman presented with a 7-month history of edema and progressive induration of the bilateral legs and forearms, associated with functional limitation due to joint contractures. Prior to presentation, these findings prompted treatment for presumptive diagnoses of congestive heart failure, lymphedema, and a parasitic infection, all without improvement. Physical examination demonstrated edema and induration in all extremities, with sparing of the distal digits. There was no associated Raynaud phenomenon or nailfold capillary changes. Linear depressions along the course of veins, better seen with limb elevation, were present on the forearms (Figure, A) and legs (Figure, B), consistent with the groove sign. The peripheral eosinophil count was 1980 cells/μL. Magnetic resonance imaging demonstrated fascial enhancement in all extremities.These findings were diagnostic of eosinophilic fasciitis (EF), a rare sclerosing disorder of the fascia that leads to erythema, edema, and induration of the extremities. Functional limitation due to joint contractures often occurs. Given its rarity, EF is often initially misdiagnosed, delaying treatment and worsening outcomes. 1, 2 Identification of the groove sign, which is characteristic