Trüeb R M, Lübbe J, Torricelli R, Panizzon R G, Wüthrich B, Burg G
Department of Dermatology, University Hospital of Zurich, Switzerland.
Arch Dermatol. 1997 Feb;133(2):203-6.
Peripheral and tissue eosinophilia are associated with a group of idiopathic inflammatory syndromes. The idiopathic hypereosinophilic syndrome represents a spectrum of disorders characterized by prolonged eosinophilia of an undetectable cause and significant organ dysfunction. The pathogenic role of the eosinophil in these conditions is attested to by evidence of eosinophil activation and degranulation at sites of tissue injury. Recently, an overlapping range of idiopathic eosinophilic muscle disease with an overall good prognosis has been described.
We describe a patient with a syndrome of idiopathic myositis with eosinophilia and eosinophilic cellulitislike cutaneous manifestations. Histopathological studies of the skin and muscle revealed eosinophilic infiltration. Elevated serum levels of eosinophilic cationic protein and interleukin-5 paralleling disease activity were detected.
This patient demonstrates clinical and laboratory features of eosinophilic myositis with eosinophilic cellulitislike skin lesions. The elevated serum levels of interleukin-5 and eosinophilic cationic protein may be responsible for the eosinophilia and tissue injury, respectively. With the advances in our understanding of cytokine-dependent regulatory mechanisms governing the eosinophil reaction, more targeted ways of manipulating eosinophilia as well as the entry and activation of eosinophils within specific tissues can be expected.
外周血和组织嗜酸性粒细胞增多与一组特发性炎症综合征相关。特发性高嗜酸性粒细胞综合征代表了一系列疾病，其特征为原因不明的持续性嗜酸性粒细胞增多以及显著的器官功能障碍。嗜酸性粒细胞在这些疾病中的致病作用通过组织损伤部位嗜酸性粒细胞活化和脱颗粒的证据得以证实。最近，已描述了一系列总体预后良好的特发性嗜酸性粒细胞性肌病。
我们描述了一名患有特发性肌炎综合征伴嗜酸性粒细胞增多及嗜酸性蜂窝织炎样皮肤表现的患者。皮肤和肌肉的组织病理学研究显示有嗜酸性粒细胞浸润。检测到血清嗜酸性粒细胞阳离子蛋白水平和白细胞介素-5水平升高，且与疾病活动度平行。
该患者表现出嗜酸性粒细胞性肌炎伴嗜酸性蜂窝织炎样皮肤病变的临床和实验室特征。血清白细胞介素-5和嗜酸性粒细胞阳离子蛋白水平升高可能分别是嗜酸性粒细胞增多和组织损伤的原因。随着我们对调控嗜酸性粒细胞反应的细胞因子依赖性调节机制理解的进展，有望出现更有针对性的方法来控制嗜酸性粒细胞增多以及嗜酸性粒细胞在特定组织内的浸润和活化。
