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[嗜酸性筋膜炎——当前认知]

[Eosinophilic fasciitis--current knowledge].

作者信息

Wojas-Pelc Anna, Wielowieyska-Szybińska Dorota, Lipko-Godlewska Sylwia

机构信息

Katedra i Klinika Dermatologii Collegium Medicum Uniwersytetu Jagiellońskiego w Krakowie, 31-501 Kraków, ul. Kopernika 19.

出版信息

Przegl Lek. 2003;60(2):116-9.

Abstract

First time eosinophilic fasciitis (EF) was described by Shulman. It occurrences predominantly in young men, rarely older women, sporadic in children. The illness was characterised by swelling of the extremities, skin changes, myalgia, and elevation of the peripheral blood eosinophil count and hypergammaglobulinemia. It is followed by rapidly progressive cutaneous and subcutaneous induration. The histopathologic examination in the region of affected skin shows thickening of the fascia, deep dermal fibrosis and infiltration mainly consists of lymphocytes, plasmocytes, eosinphils. It is suggested that the cells from infiltration induce fibrosis. The tryptophan disturbance has significant influence on development of EF in predisposed persons. Eosinophilic fasciitis is classified to wide spectrum of scleroderma disorders by many authors. The authors tried to show differences between EF, morphea and systemic scleroderma.

摘要

首次由舒尔曼描述了嗜酸性筋膜炎(EF)。它主要发生在年轻男性中,老年女性很少见,儿童中则为散发性。该病的特征是四肢肿胀、皮肤改变、肌痛、外周血嗜酸性粒细胞计数升高和高球蛋白血症。随后会出现迅速进展的皮肤和皮下硬结。在受影响皮肤区域的组织病理学检查显示筋膜增厚、真皮深层纤维化,浸润主要由淋巴细胞、浆细胞、嗜酸性粒细胞组成。提示浸润细胞诱导纤维化。色氨酸紊乱对易感人群中EF的发展有重大影响。许多作者将嗜酸性筋膜炎归类为硬皮病疾病的广泛谱系。作者试图展示EF、硬斑病和系统性硬皮病之间的差异。

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