Eosinophilic fasciitis and eosinophilic cellulitis in a patient with abnormal circulating clonal T cells: increased production of interleukin 5 and inhibition by interferon alfa
Eosinophilic fasciitis and eosinophilic cellulitis in a patient with abnormal circulating clonal T cells: increased production of interleukin 5 and inhibition by interferon alfa
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DOI：
10.1016/s0190-9622(03)00447-x
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发表时间：
2003-12-01
影响因子：
13.8
通讯作者：
Rook, AH
中科院分区：
文献类型：
--
作者：
French, LE;Shapiro, M;Rook, AH
嗜酸性筋膜炎(舒尔曼综合征)和嗜酸性蜂窝织炎是以组织和外周血嗜酸性粒细胞增多为特征的一系列疾病的一部分。嗜酸性粒细胞直接参与了表征这些情况的皮损过程，因为在组织损伤部位观察到嗜酸性粒细胞激活和脱颗粒的迹象。嗜酸性筋膜炎和蜂窝织炎的病因和发病机制目前尚不清楚。在此，我们报告了一位患者，表现为手臂和腿部快速进行性局限性皮肤硬化，伴有嗜酸性粒细胞增多症，没有系统性硬化症的迹象，组织病理学特征与嗜酸性筋膜炎的诊断相一致。嗜酸性筋膜炎发病四年后，患者反复发作嗜酸性蜂窝织炎。对克隆性T细胞受体γ基因重排的血液筛查显示有几个循环T细胞的克隆性扩增群体。此外，对患者外周血单个核细胞产生的细胞因子进行的体外分析显示，白介素5的产生显著增加，而白介素5的合成可被干扰素-α完全阻断。在循环T细胞克隆异常和IL-5产生增加的患者中，嗜酸性筋膜炎和蜂窝织炎共存是独一无二的，这可能是嗜酸性粒细胞增多和嗜酸性粒细胞介导的组织损伤的原因。虽然在这位患者身上没有进行体内评估，但我们的体外数据为使用干扰素-α治疗嗜酸性筋膜炎和/或蜂窝织炎提供了理论依据。
Eosinophilic fasciitis (Shulman's syndrome) and eosinophilic cellulitis are part of a spectrum of diseases characterized by tissue and peripheral blood eosinophilia. Eosinophils are implicated directly in the lesional process that characterizes these conditions, because signs of eosinophil activation and degranulation are observed at the sites of tissue injury. The cause and pathogenesis of eosinophilic fasciitis and cellulitis are presently unclear. Herein, we report a patient manifesting rapidly progressive localized cutaneous induration of the arms and legs with eosinophilia, no signs of systemic sclerosis, and histopathologic features compatible with the diagnosis of eosinophilic fasciitis. Four years after the onset of eosinophilic fasciitis, the patient had recurrent episodes of eosinophilic cellulitis. Blood screening for clonal T-cell receptor gamma gene rearrangements revealed several amplified clonal populations of circulating T cells. Furthermore, in vitro analysis of cytokine production by the patient's peripheral blood mononuclear cells demonstrated strongly increased production of interleukin 5, the synthesis of which Could be completely blocked by interferon (IFN)-alpha. The coexistence of eosinophilic fasciitis and cellulitis in a patient with an abnormal circulating T-cell clone and increased IL-5 production are unique and might be responsible for the eosinophilia and eosinophil-mediated tissue injury. Although not assessed in vivo in this patient, our in vitro data provide a rationale for the use of IFN-alpha in eosinophilic fasciitis and/or cellulitis.