Intravascular crystal deposition: an early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.

Intravascular crystal deposition: an early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.
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血管内晶体沉积:诊断 1 型冷球蛋白血症性血管炎的早期线索。

DOI:
10.1097/dad.0b013e31829ff8d9
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发表时间:
2014
期刊:
The American Journal of dermatopathology
影响因子:
--
通讯作者:
DeClerck,Brittney
DeClerck,Brittney
中科院分区:
--
文献类型:
--
作者:
Gammon,Bryan;Longmire,Michelle;DeClerck,Brittney

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皮肤小血管炎(CSVV)是一种非特异性的表现,具有广泛的鉴别诊断。区分CSVV的皮肤受限表现和以CSVV为首发表现的严重危及生命的全身性血管炎是至关重要的。确定哪些CSVV患者有患全身性疾病的风险可能是具有挑战性的。通常需要标准的组织病理学评估、直接免疫荧光和血清学评估来排除系统性脉管炎。1型冷球蛋白血症很少出现在CSVV中。在此,我们报告一例隐匿性多发性骨髓瘤的1型冷球蛋白血症。CSVV有明显的血管内晶体形成。CSVV内血管内晶体的存在可能是诊断1型冷球蛋白血症小血管炎的重要早期线索。
Cutaneous small vessel vasculitis (CSVV) is a nonspecific finding with an extensive differential diagnosis. It is critically important to distinguish skin-limited presentations of CSVV from severe life-threatening systemic vasculitides presenting with CSVV as an initial manifestation. It can be challenging to determine which patients presenting with CSVV are at risk for systemic disease. Standard histopathologic evaluation, direct immunofluorescence, and serologic evaluation is typically required to exclude a systemic vasculitis. Type 1 cryoglobulinemia may rarely present with CSVV. Herein, we report a case of type 1 cryoglobulinemia in the setting of occult multiple myeloma. CSVV with prominent intravascular crystal formation was noted. The presence of intravascular crystals in the setting of CSVV may represent an important early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.
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