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[血小板减少作为急性播散性红斑狼疮的首发症状:Moschcowitz病]

[Thrombopenia as the presenting symptom of acute disseminated lupus erythematosus: Moschcowitz's disease].

作者信息

Bourrier P, Cousteau C, Gounant C, Grodner F, Ruf R, Szpirglas H, Texier J, Laufer J

出版信息

Rev Stomatol Chir Maxillofac. 1983;84(4):196-8.

PMID:6579593
Abstract

Possible relationships between Moschcowitz's disease and acute systemic lupus erythematosus are analyzed based on findings in one case. Initial exclusively buccal symptoms suggested a diagnosis of lupus, and then of Moschcowitz's disease leading to a fatal outcome. The main points discussed relate to diagnostic difficulties to enable distinction between the two diseases. A thrombotic thrombopenic purpura during the second phase of the disease showed a characteristic microangiopathy associated with a non-autoimmune hemolytic anemia and a schizocytosis: two forms of the affection are recognized, one with a chronic and one with an acute course. The latter, which has a fulminating progression leading to death, was that described in the case reported.

摘要

基于一个病例的研究结果,分析了血栓性血小板减少性紫癜(Moschcowitz病)与急性系统性红斑狼疮之间可能存在的关系。最初仅出现颊部症状,提示诊断为狼疮,之后又诊断为Moschcowitz病,最终导致死亡。讨论的要点涉及诊断困难,以便能够区分这两种疾病。疾病第二阶段出现的血栓性血小板减少性紫癜表现出特征性微血管病,伴有非自身免疫性溶血性贫血和红细胞碎片形成:该病有两种形式,一种为慢性病程,另一种为急性病程。后者病情呈暴发性进展并导致死亡,正是报告病例中所描述的情况。

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