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[嗜酸性筋膜炎——向一种罕见硬皮病(硬皮病-筋膜炎)的转变]

[Fasciitis eosinophilica--transition to an unusual scleroderma (scleroderma-fasciitis)].

作者信息

Jablonska S, Hamm G, Kencka D, Sieminska S

出版信息

Z Hautkr. 1984 Jun 1;59(11):711-21.

PMID:6485447
Abstract

Eosionophilic fasciitis (EF) was observed in a girl who suddenly developed indurations of the limbs accompanied by hypergammaglobulinaemia and peripheral eosinophilia. The deep fascia was impressively thickened and infiltrated with numerous inflammatory cells showing focal accumulations of eosinophils. The disease was progressive in spite of application of corticosteroids, and within three years, symmetrical sclerodermatous lesions developed on the face and trunk, going along with a severe sclerodactyly without Raynaud's phenomenon, as well as contractures of the upper and lower limbs causing complete disability. Visceral involvement was slight (decreased motility of the esophagus and restriction of pulmonal function); slight calcium deposits were disclosed at the soft tissue of the ankles; and nuclear antibodies of the speckled type appeared in a titer up to 320. The fascia was still the most involved tissue, but there was no peripheral or tissue eosinophilia. EF seems to be a special variant within the broad spectrum of scleroderma; and although it usually has a benign course, it may change into an unclassified variety of scleroderma and lead to severe disability.

摘要

在一名突然出现肢体硬结并伴有高球蛋白血症和外周血嗜酸性粒细胞增多的女孩中观察到嗜酸性筋膜炎(EF)。深筋膜明显增厚,有大量炎症细胞浸润,可见嗜酸性粒细胞灶性聚集。尽管应用了皮质类固醇,病情仍在进展,三年内,面部和躯干出现对称性硬皮病样病变,同时伴有严重的指(趾)硬皮病但无雷诺现象,以及上肢和下肢挛缩导致完全残疾。内脏受累较轻(食管蠕动减弱和肺功能受限);踝关节软组织有轻微钙沉积;斑点型核抗体滴度高达320。筋膜仍是受累最严重的组织,但外周血或组织中无嗜酸性粒细胞增多。EF似乎是硬皮病广泛谱系中的一种特殊变体;尽管它通常病程良性,但可能转变为未分类的硬皮病类型并导致严重残疾。

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