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静脉注射免疫球蛋白治疗皮肤白细胞碎裂性血管炎:临床疗效和患者活动水平的回顾性观察。

Intravenous Immunoglobulin Therapy in Livedoid Vasculopathy: Retrospective Observation of Clinical Outcome and Patient's Activity Level.

机构信息

9188 Department of Dermatology, Eberhard-Karls University, Tübingen, Germany.

出版信息

J Cutan Med Surg. 2021 Sep;25(5):504-510. doi: 10.1177/12034754211003525. Epub 2021 Mar 28.

Abstract

BACKGROUND

Livedoid vasculopathy (LV) is a rare disease characterized by livedo racemosa, atrophie blanche, ulcerations, and severe pain. Low molecular weight heparins and rivaroxaban can be used in LV-patients. In addition, intravenous immunoglobulins (IVIG) have been described as treatment-option.

OBJECTIVES

Objective was to investigate the therapeutic effect of IVIG on ulcer, pain and restrictions in daily life.

METHODS

Thirty-two LV-patients who received IVIG at the Department of Dermatology Tübingen between 01/2014 and 06/2019 were identified. Twenty-five of these patients were available for further follow up and were included in the study. Patients were interviewed using a questionnaire focusing on the course of the disease, symptoms, and subjective response to IVIG-treatment.

RESULTS

Twenty-five patients were included in the study (mean follow up: 28.9 months). Patients received an average of 6.8 cycles (range 1-45) of IVIG during the observed period.Significant improvements were seen regarding skin findings, pain, and limitation of daily activities. Complete remission of symptoms was observed in 68% of patients. Good tolerability of IVIG was shown in 92%.

CONCLUSIONS

A good therapy response regarding ulceration, pain, and daily life restrictions with good tolerability was demonstrated for IVIG (2 g/kg bodyweight over 5 days).

摘要

背景

皮肤白细胞碎裂性血管炎(LV)是一种罕见疾病,其特征为匐行性青斑、白萎缩、溃疡和剧烈疼痛。低分子肝素和利伐沙班可用于 LV 患者。此外,静脉注射免疫球蛋白(IVIG)也被描述为一种治疗选择。

目的

本研究旨在探讨 IVIG 对溃疡、疼痛和日常生活受限的治疗效果。

方法

本研究回顾性分析了 2014 年 1 月至 2019 年 6 月在图宾根皮肤科接受 IVIG 治疗的 32 例 LV 患者。其中 25 例患者可进行进一步随访,并纳入本研究。通过问卷调查的方式对患者进行访谈,重点关注疾病的病程、症状以及对 IVIG 治疗的主观反应。

结果

本研究共纳入 25 例患者(平均随访时间:28.9 个月)。在观察期间,患者平均接受了 6.8 个周期(范围 1-45 个)的 IVIG 治疗。皮肤表现、疼痛和日常生活受限均有显著改善。68%的患者症状完全缓解。92%的患者对 IVIG 具有良好的耐受性。

结论

IVIG(2g/kg 体重,连用 5 天)治疗皮肤白细胞碎裂性血管炎具有良好的耐受性,可显著改善溃疡、疼痛和日常生活受限等症状。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fa5c/8474298/e6f312e15c99/10.1177_12034754211003525-fig1.jpg

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Quality-of-life impairment in patients with livedoid vasculopathy.萎缩性硬化性苔藓样血管病患者的生活质量损害
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