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成人特发性皮肤小血管炎的处理

Author
Nicole Fett, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD
Translators
罗晓燕, 副主任医师，副教授

引言

皮肤小血管炎(cutaneous small vessel vasculitis, CSVV)是一种以累及皮肤小血管的白细胞碎裂性血管炎为特征的疾病。顾名思义，该病一定不存在累及其他器官系统的血管炎。CSVV发病可能与多种因素相关，包括药物、感染、全身性疾病或恶性肿瘤。约1/3-1/2患者无明确诱因，在此，我们将这类患者归为特发性CSVV。

特发性CSVV常在数周内缓解，因此，大多数患者的处理包括证明无可识别病因和缓解症状。用于阻止疾病进展的全身性免疫调节治疗通常仅用于有并发症的患者，如出血性大疱、溃疡或者慢性或复发性病变。

特发性CSVV患者的处理将总结在此。血管炎性皮肤病变患者的评估，以及可能表现为皮肤小血管受累的血管炎的特定疾病的临床特征和治疗将单独讨论。(参见“成人血管炎性皮肤病变的评估”和“成人过敏性血管炎”和“荨麻疹性血管炎”和“过敏性紫癜(IgA血管炎)的治疗”和“类风湿血管炎的治疗”和“Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)”和“肉芽肿性多血管炎和显微镜下多血管炎的免疫抑制初始治疗”和“Treatment of the mixed cryoglobulinemia syndrome”)

专业术语

其他被用于指代CSVV的术语包括皮肤白细胞碎裂性血管炎(cutaneous leukocytoclastic angiitis和cutaneous necrotizing venulitis)及皮肤坏死性小静脉炎，这些术语都是指CSVV的特征性组织病理学表现。过敏性血管炎这一术语也已被用来指代CSVV，但它最常用于由已知药物或感染所致的CSVV。(参见“成人血管炎性皮肤病变的评估”，关于‘专业术语’一节和“成人过敏性血管炎”)

临床特征及评估

CSVV的特点是存在皮肤病变，同时还存在累及皮肤毛细血管、小静脉和小动脉的白细胞碎裂性血管炎的组织病理学证据。尽管可触性紫癜是最常见的伴随皮肤表现，但其他皮损如瘀点、出血性大疱、溃疡和荨麻疹也可能发生(图片 1A-G)。皮损最常发生于小腿及其他重力依赖区。

             

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Literature review current through: 2017-06 . | This topic last updated: 2017-04-17.
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