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嗜酸性筋膜炎

Eosinophilic fasciitis.

作者信息

Sibrack L A, Mazur E M, Hoffman R, Bollet A J

出版信息

Clin Rheum Dis. 1982 Aug;8(2):443-54.

PMID:6754236
Abstract

Eosinophilic fasciitis (EF, a diffuse fasciitis with eosinophilia) is a recently recognized connective tissue disorder. It consists of deeply indurated, bound-down plaques of skin and subcutaneous tissue, most commonly present on the extremities. It is associated with peripheral eosinophilia, hypergammaglobulinaemia and an elevated sedimentation rate. There is usually no evidence of Raynaud's phenomenon, acral sclerosis or visceral involvement. Antinuclear antibodies are usually absent. The abnormal histopathology primarily involves the lower subcutis and the fascia. The clinical and laboratory features usually improve following the administration of systemic corticosteroids. Serious haematological abnormalities have been associated with eosinophilic fasciitis (EF) and have been discussed in detail. The similarities and distinctions between eosinophilic fasciitis and scleroderma have been discussed.

摘要

嗜酸性筋膜炎(EF,一种伴有嗜酸性粒细胞增多的弥漫性筋膜炎)是一种最近才被认识的结缔组织疾病。它由皮肤和皮下组织的深部硬结、粘连斑块组成,最常见于四肢。它与外周嗜酸性粒细胞增多、高球蛋白血症和血沉升高有关。通常没有雷诺现象、肢端硬化或内脏受累的证据。抗核抗体通常不存在。异常的组织病理学主要累及皮下深层和筋膜。全身使用糖皮质激素后,临床和实验室特征通常会改善。严重的血液学异常与嗜酸性筋膜炎(EF)有关,并已进行了详细讨论。嗜酸性筋膜炎和硬皮病之间的异同也已进行了讨论。

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