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家族性地中海热中的脂膜炎。病例报告及组织病理学发现

Panniculitis in familial Mediterranean fever. Case report with histopathologic findings.

作者信息

Danar D A, Kwan T H, Stern R S, Kasdon E J, Birnbaum P S, Brown R S

出版信息

Am J Med. 1987 Apr;82(4):829-32. doi: 10.1016/0002-9343(87)90023-4.

DOI:10.1016/0002-9343(87)90023-4
PMID:3565436
Abstract

A 79-year-old Armenian-born woman with stable, long-term familial Mediterranean fever had progression of chronic renal failure concurrently with two types of skin lesions. One lesion resembled erysipelas, which is quite common in familial Mediterranean fever, whereas the other was panniculitis, only occasionally described in familial Mediterranean fever. The unique histopathologic features of the latter are presented. The onset of acute cutaneous disease in this patient coincided with worsening renal disease and preceded a flare of disease activity in other sites after a 14-year period of quiescence. The severe and unremitting pain from the skin lesions and their tendency to form nonhealing ulcers were substantial causes of morbidity in this patient with familial Mediterranean fever.

摘要

一名79岁出生于亚美尼亚的女性,患有稳定的长期家族性地中海热,同时出现慢性肾衰竭进展并伴有两种皮肤病变。一种病变类似丹毒,这在家族性地中海热中相当常见,而另一种是脂膜炎,在家族性地中海热中仅偶尔有描述。本文展示了后者独特的组织病理学特征。该患者急性皮肤疾病的发作与肾病恶化同时出现,并在14年的静止期后先于其他部位的疾病活动发作。皮肤病变引起的剧烈且持续的疼痛以及它们形成不愈合溃疡的倾向,是该家族性地中海热患者发病的主要原因。

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Is bullous skin lesion a risk factor for renal amyloidosis in patients with familial mediterranean fever?大疱性皮肤病变是家族性地中海热患者肾淀粉样变性的危险因素吗?
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