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淀粉样血管病:在一例合并潜在骨髓瘤的病例中出现黏膜皮肤动脉索状增厚、间歇性跛行和心绞痛。

Amyloid vascular disease: cord-like thickening of mucocutaneous arteries, intermittent claudication and angina in a case with underlying myelomatosis.

作者信息

Breathnach S M, Wells G C

出版信息

Br J Dermatol. 1980 May;102(5):591-5. doi: 10.1111/j.1365-2133.1980.tb07661.x.

Abstract

A patient with established myelomatosis and an IgG paraproteinaemia presented with intermittent claudication. Indurated cord-like structures were noted on the dorsum of the right hand and the buccal surface of the lower lip, and biopsy of the hand lesion revealed massive amyloid deposition within the wall of an artery. Cord-like thickening of mucocutaneous arteries as a presenting sign of systemic amyloidosis in the absence of glossomegaly or other cutaneous lesions has not been reported previously. The presence of intermittent claudication and angina suggested that amyloid involvement of large arteries was extensively distributed.

摘要

一位患有多发性骨髓瘤和IgG副蛋白血症的患者出现间歇性跛行。右手背部和下唇颊面可见硬结条索状结构,手部病变活检显示动脉壁内有大量淀粉样蛋白沉积。黏膜皮肤动脉的条索状增厚作为系统性淀粉样变性的首发体征,且无巨舌或其他皮肤病变,此前尚未见报道。间歇性跛行和心绞痛的存在提示大动脉的淀粉样蛋白累及广泛分布。

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