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一例罕见病例,最初表现为皮下脂膜炎,随后出现发热、严重肝脏受累及高脂血症。

An unusual case which began with subcutaneous panniculitis followed by fever, severe hepatic involvement and hyperlipidemia.

作者信息

Miyasaki K, Ooiso Y, Nakamura I, Oimomi M, Tai T, Saito K

出版信息

Acta Pathol Jpn. 1977 Mar;27(2):213-24. doi: 10.1111/j.1440-1827.1977.tb01870.x.

Abstract

A 25-year-old man was at first dermatologically suspected as suffering from Weber-Christian syndrome because of subcutaneous panniculitis, but his skin lesions disappeared completely during the course. Hyperlipidemia, disturbances in liver function, and leukemoid reaction became remarkable and he died of subarachnoid hemorrhage eleven months after onset. Necropsy revealed subarachnoid hemorrhage at the base of the brain, lipogranulomatous and inflammatory lesions in the upper lobe of the left lung, a remarkable fatty liver, splenomegaly, pericarditis, and foam cells in the spleen, liver, and bone marrow. A comparison with 57 autopsy cases of Weber-Christian syndrome reported in the literature showed our case to be an exceptional instance of Weber-Christian syndrome, if the present case is not to be regarded as a different disease entity.

摘要

一名25岁男性起初因皮下脂膜炎在皮肤科被怀疑患有韦格纳-克里斯蒂安综合征,但在病程中其皮肤病变完全消失。高脂血症、肝功能紊乱和类白血病反应变得明显,发病11个月后他死于蛛网膜下腔出血。尸检发现脑底部蛛网膜下腔出血、左肺上叶脂肉芽肿性和炎症性病变、明显的脂肪肝、脾肿大、心包炎以及脾脏、肝脏和骨髓中的泡沫细胞。与文献报道的57例韦格纳-克里斯蒂安综合征尸检病例相比,如果不将本病例视为一种不同的疾病实体,那么我们的病例就是韦格纳-克里斯蒂安综合征的一个特殊实例。

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