9188 Department of Dermatology, Eberhard-Karls University, Tübingen, Germany.
J Cutan Med Surg. 2021 Sep;25(5):504-510. doi: 10.1177/12034754211003525. Epub 2021 Mar 28.
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.
Objective was to investigate the therapeutic effect of IVIG on ulcer, pain and restrictions in daily life.
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.
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%.
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).
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。
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。
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。
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。
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。
