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白塞病中膜性囊肿样病变(那须氏)样改变的出现。结节性红斑样病变的电子显微镜研究。

Appearance of membranocystic lesion (Nasu)-like changes in Behçet's syndrome. An electron microscopic study of erythema nodosum-like lesions.

作者信息

Honma T, Bang D, Saito T, Nakagawa S, Ueki H, Lee S

机构信息

Department of Pharmacology, Kawasaki Medical School, Kurashiki, Japan.

出版信息

Acta Pathol Jpn. 1988 Aug;38(8):1001-10. doi: 10.1111/j.1440-1827.1988.tb02372.x.

DOI:10.1111/j.1440-1827.1988.tb02372.x
PMID:3188908
Abstract

Membranocystic lesion (Nasu)-like changes were seen in the subcutaneous fat tissues of erythema nodosum-like lesions in three of 18 patients with Behçet's syndrome. The membranocystic structures, which were derived from degenerative fat cells with a loss of the cell membrane, were observed in the edematous connective tissues accompanied by fibrin deposition and diapedesis of erythrocytes. Electron microscopic evaluation revealed two different patterns of peculiar membranous structures in the cytoplasm of the degenerative fat cells. One was proliferating membrane structures such as well developed Golgi-endoplasmic reticulum, and the other was minute tubular structures such as intracytoplasmic microvesicles. These degenerative fat cells are considered to be derived from abnormal fat cells which lack a cell membrane, but whose cellular and nuclear configurations appear to be normal morphologically, because the cytoplasm of these abnormal cells showed the same tubular structures as those seen in the characteristic architecture of membranocystic lesion-like change. The relation between the appearance of such abnormal cells and the pathologic conditions of erythema nodosum-like lesions in Behçet's syndrome was discussed.

摘要

在18例白塞病患者中,有3例患者结节性红斑样病变的皮下脂肪组织出现了膜性囊肿样(纳苏)改变。在伴有纤维蛋白沉积和红细胞渗出的水肿性结缔组织中,观察到了由细胞膜缺失的变性脂肪细胞形成的膜性囊肿结构。电子显微镜评估显示,变性脂肪细胞的细胞质中有两种不同类型的特殊膜性结构。一种是增殖性膜结构,如发育良好的高尔基体-内质网;另一种是微小管状结构,如胞质内微泡。这些变性脂肪细胞被认为源自缺乏细胞膜但细胞和细胞核形态在形态学上看似正常的异常脂肪细胞,因为这些异常细胞的细胞质显示出与膜性囊肿样改变特征结构中所见相同的管状结构。讨论了此类异常细胞的出现与白塞病结节性红斑样病变病理状况之间的关系。

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Appearance of membranocystic lesion (Nasu)-like changes in Behçet's syndrome. An electron microscopic study of erythema nodosum-like lesions.白塞病中膜性囊肿样病变(那须氏)样改变的出现。结节性红斑样病变的电子显微镜研究。
Acta Pathol Jpn. 1988 Aug;38(8):1001-10. doi: 10.1111/j.1440-1827.1988.tb02372.x.
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Virchows Arch A Pathol Anat Histopathol. 1988;413(6):475-83. doi: 10.1007/BF00750387.

引用本文的文献

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Nasu-Hakola syndrome: polycystic lipomembranous osteodysplasia with sclerosing leucoencephalopathy and presenile dementia.那须-哈科拉综合征:伴硬化性白质脑病和早老性痴呆的多囊性脂膜性骨发育异常
J Med Genet. 1997 Sep;34(9):753-7. doi: 10.1136/jmg.34.9.753.