Falanga V, Medsger T A
University of Miami School of Medicine, Department of Dermatology and Cutaneous Surgery, FL 33101.
J Am Acad Dermatol. 1987 Oct;17(4):648-56. doi: 10.1016/s0190-9622(87)70251-5.
Blood eosinophilia is a common feature of eosinophilic fasciitis and is variably reported in systemic sclerosis and localized scleroderma. Since these diseases share cutaneous fibrosis as the final outcome and have other clinical and pathologic features that are difficult to differentiate, the presence of blood eosinophilia may be a further source of confusion. In this study, we examined the frequency and level of blood eosinophilia in 715 patients with systemic sclerosis, 72 patients with localized scleroderma, and 22 patients with clinically active eosinophilic fasciitis. When defined as greater than 400 cells/mm3, eosinophilia was present in 7% of patients with systemic sclerosis, 31% of patients with localized scleroderma, and 83% of patients with eosinophilic fasciitis. Greater than 1000 eosinophils/mm3 were present less frequently in systemic sclerosis (1%) and localized scleroderma (8%) than in eosinophilic fasciitis (61%). No difference in the frequency of eosinophilia was present in patients with the limited cutaneous CREST syndrome or the diffuse cutaneous variety of systemic sclerosis, and in these patients the presence of eosinophilia did not correlate with the extent of cutaneous or internal organ involvement or with other laboratory abnormalities. Among patients with localized scleroderma, eosinophilia was more common in those with linear scleroderma and generalized morphea than in those with morphea, and both the frequency and level of eosinophilia were greater in individuals with clinically active disease (p less than 0.02). Eosinophilia was a persistent feature in untreated patients with active eosinophilic fasciitis, even up to 30 months of disease duration.
血液嗜酸性粒细胞增多是嗜酸性筋膜炎的常见特征，在系统性硬化症和局限性硬皮病中也有不同程度的报道。由于这些疾病最终都会出现皮肤纤维化，且具有其他难以区分的临床和病理特征，血液嗜酸性粒细胞增多的存在可能会进一步造成混淆。在本研究中，我们检测了715例系统性硬化症患者、72例局限性硬皮病患者和22例临床活动期嗜酸性筋膜炎患者的血液嗜酸性粒细胞增多的频率和水平。当定义为大于400个细胞/mm³时，系统性硬化症患者中有7%出现嗜酸性粒细胞增多，局限性硬皮病患者中有31%出现，嗜酸性筋膜炎患者中有83%出现。系统性硬化症（1%）和局限性硬皮病（8%）中嗜酸性粒细胞大于1000/mm³的情况比嗜酸性筋膜炎（61%）中少见。局限性皮肤CREST综合征或系统性硬化症弥漫性皮肤型患者的嗜酸性粒细胞增多频率没有差异，在这些患者中，嗜酸性粒细胞增多的存在与皮肤或内脏器官受累程度或其他实验室异常无关。在局限性硬皮病患者中，嗜酸性粒细胞增多在线状硬皮病和泛发性硬斑病患者中比在硬斑病患者中更常见，且在临床活动期疾病患者中嗜酸性粒细胞增多的频率和水平都更高（p小于0.02）。嗜酸性粒细胞增多是未经治疗的活动期嗜酸性筋膜炎患者的一个持续特征，甚至在疾病持续长达30个月时也是如此。
