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非典型胶原病。多发性肌炎、显著的苏木精小体形成以及进行性系统性硬化症的一些表现。

Atypical collagen disease. Polymyositis, prominent hematoxylin body formation and some manifestations of progressive systemic sclerosis.

作者信息

Mitsuhashi T, Hojo K, Kanayama Y

出版信息

Acta Pathol Jpn. 1980 Nov;30(6):1019-35. doi: 10.1111/j.1440-1827.1980.tb03288.x.

DOI:10.1111/j.1440-1827.1980.tb03288.x
PMID:7446115
Abstract

An autopsy case of polymyositis with prominent hematoxylin body formation in various organs and tissues, florid focal and segmental necrotizing glomerulonephritis, and some manifestations of progressive systemic sclerosis was presented. A 34-year-old woman had erythematous edema on the face, joint pain, muscular weakness, cutaneous sclerosis and Raynaud's phenomenon for about 5 years. She had also a long history of pulmonary tuberculosis. Autopsy revealed polymyositis involving skeletal, smooth and heart muscles. This was widespread consisting of focal and extensive primary degeneration of muscle fibers and interstitial inflammatory cell infiltrates near and surrounding small veins and venules accompanied by the derivation of hematoxylin bodies from inflammatory cell nuclei, and in the posterior pharyngeal muscles severely affected, muscle substance was literally erased, leaving interstitial fibrous tissues and blood vessels. This focally restricted, severe necrosis of the skeletal muscle gives a special feature to the polymyositis in the present case. The diagnosis of polymyositis with prominent hematoxylin body formation and some manifestations of progressive systemic sclerosis was applied as a rather descriptive designation, but yet it does not absolutely exclude the possibility of a variant of SLE in an additional effort to search for an underlying disorder.

摘要

本文报告了一例多肌炎尸检病例,该病例在多个器官和组织中出现了显著的苏木精小体形成、弥漫性局灶性和节段性坏死性肾小球肾炎以及一些进行性系统性硬化症的表现。一名34岁女性面部出现红斑性水肿、关节疼痛、肌肉无力、皮肤硬化和雷诺现象约5年。她还有长期的肺结核病史。尸检显示多肌炎累及骨骼肌、平滑肌和心肌。病变广泛,包括肌纤维的局灶性和广泛性原发性变性,以及小静脉和微静脉周围及附近的间质炎性细胞浸润,伴有炎性细胞核衍生出苏木精小体,在严重受累的咽后肌中,肌组织几乎完全消失,仅留下间质纤维组织和血管。这种骨骼肌的局灶性局限性严重坏死是本例多肌炎的一个特殊特征。诊断为具有显著苏木精小体形成和一些进行性系统性硬化症表现的多肌炎,这是一个较为描述性的诊断,但在进一步寻找潜在疾病时,也不能完全排除系统性红斑狼疮变体的可能性。

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Atypical collagen disease. Polymyositis, prominent hematoxylin body formation and some manifestations of progressive systemic sclerosis.非典型胶原病。多发性肌炎、显著的苏木精小体形成以及进行性系统性硬化症的一些表现。
Acta Pathol Jpn. 1980 Nov;30(6):1019-35. doi: 10.1111/j.1440-1827.1980.tb03288.x.
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