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嗜酸性粒细胞增多预示嗜酸性筋膜炎(舒尔曼病)的诊断

[Eosinophilia heralding the diagnosis of eosinophilic fasciitis (Shulman's disease)].

作者信息

Ernest V, Sautereau N, Masson E, Chemouni D, Garcia M, Bertolino J, Rossi P, Granel B

机构信息

Service de médecine interne, hôpital Nord, Aix-Marseille université, Assistance publique-Hôpitaux de Marseille (AP-HM), chemin des Bourrely, 13915 Marseille cedex 15, France.

Service d'imagerie médicale, hôpital Nord, Aix-Marseille université, Assistance publique-Hôpitaux de Marseille (AP-HM), chemin des Bourrely, 13915 Marseille cedex 15, France.

出版信息

Rev Med Interne. 2017 Dec;38(12):840-843. doi: 10.1016/j.revmed.2017.07.010. Epub 2017 Sep 1.

Abstract

INTRODUCTION

Eosinophilic fasciitis or Shulman's disease is characterized, in its typical form, by palpable thickening of the skin and soft tissues, blood hypereosinophilia and fascia lesions. We hereby report a case of eosinophilic fasciitis in which hypereosinophilia preceded for several months the clinical signs of fasciitis.

CASE REPORT

A 64-year-old woman, with a history of Little's syndrome with motor disability, was admitted in internal medicine for eosinophilia. For almost three months, no origin to the eosinophilia was found. The secondary onset of an edema and pain located on four limbs led to the diagnosis of eosinophilic fasciitis. Muscle magnetic resonance imaging was supportive and the muscle histological analysis confirmed the diagnosis of eosinophilic fasciitis. Treatment with steroids induced a rapid normalization of the eosinophilia and edema.

CONCLUSION

In this case report, eosinophilia was preceding the clinical cutaneous signs that led to the diagnosis of eosinophilic fasciitis. It is likely to believe that myalgias, frequently found in the onset of eosinophilic fasciitis, may have been hidden by the history of infantile encephalopathy. The diagnosis of eosinophilic fasciitis must be kept in mind of physicians in the investigation of an eosinophilia, even though cutaneous signs are lacking.

摘要

引言

嗜酸性筋膜炎或舒尔曼病的典型表现为皮肤和软组织可触及的增厚、血液嗜酸性粒细胞增多以及筋膜病变。我们在此报告一例嗜酸性筋膜炎病例,其中嗜酸性粒细胞增多在筋膜炎临床症状出现前数月就已存在。

病例报告

一名64岁女性,有患伴有运动障碍的利特尔综合征病史,因嗜酸性粒细胞增多入住内科。近三个月来,未找到嗜酸性粒细胞增多的病因。四肢出现水肿和疼痛的继发症状后,诊断为嗜酸性筋膜炎。肌肉磁共振成像结果支持该诊断,肌肉组织学分析也证实了嗜酸性筋膜炎的诊断。使用类固醇治疗后,嗜酸性粒细胞增多和水肿迅速恢复正常。

结论

在本病例报告中,嗜酸性粒细胞增多先于导致嗜酸性筋膜炎诊断的临床皮肤症状出现。很可能是嗜酸性筋膜炎发病时常见的肌痛被婴儿期脑病病史掩盖了。即使缺乏皮肤症状,医生在调查嗜酸性粒细胞增多时也必须考虑嗜酸性筋膜炎的诊断。

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