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黏液样血管内筋膜炎

Myxoid intravascular fasciitis.

作者信息

Wang Lei, Wang Gang, Wang Lin, Gao Tianwen

机构信息

Department of Dermatology, Xijing Hospital, The Fourth Military Medical University, 15 West Changle Rd, Xian 710032, China.

出版信息

J Cutan Pathol. 2011 Jan;38(1):63-6. doi: 10.1111/j.1600-0560.2009.01445.x.

DOI:10.1111/j.1600-0560.2009.01445.x
PMID:19863703
Abstract

Nodular fasciitis is a common reactive proliferation of soft tissue. It is composed of high density of fibroblasts and myofibroblasts, and sometimes shows a sarcomatoid appearance histologically. A rare variant of nodular fasciitis, intravascular fasciitis can be found in the veins or arteries. Histologically, intravascular fasciitis has been described that resembles nodular fasciitis with a less prominent mucoid matrix. Here, we report a case of intravascular fasciitis with prominent mucin deposition. Clinically, it presented as multiple nodules on the leg of the patient. Histologically, it showed proliferation of spindle cells in a vein of the dermis and Alcian blue staining confirmed prominent mucin deposition between the spindle cells. Immunohistochemistry studies showed the spindle cells were positive for vimentin, actin and smooth muscle actin, but negative for keratin, S100 protein, desmin, CD31 and CD34. Based on the clinical and histopathological properties, a diagnosis of myxoid intravascular fasciitis was made.

摘要

结节性筋膜炎是一种常见的软组织反应性增生。它由高密度的成纤维细胞和肌成纤维细胞组成,组织学上有时表现出肉瘤样外观。结节性筋膜炎的一种罕见变体,血管内筋膜炎可在静脉或动脉中发现。组织学上,血管内筋膜炎已被描述为类似于结节性筋膜炎,黏液样基质不那么突出。在此,我们报告一例伴有显著黏蛋白沉积的血管内筋膜炎病例。临床上,它表现为患者腿部的多个结节。组织学上,它显示真皮静脉内梭形细胞增生,阿尔辛蓝染色证实梭形细胞之间有显著的黏蛋白沉积。免疫组织化学研究显示梭形细胞波形蛋白、肌动蛋白和平滑肌肌动蛋白呈阳性,但角蛋白、S100蛋白、结蛋白、CD31和CD34呈阴性。根据临床和组织病理学特征,诊断为黏液样血管内筋膜炎。

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