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伴有亚甲基四氢叶酸还原酶基因多态性的难治性类脂质渐进性坏死性血管病经高压氧治疗成功治愈

A Refractory Livedoid Vasculopathy Accompanied by Methylene Tetrahydrofolate Reductase Gene Polymorphism Successfully Treated with Hyperbaric Oxygen Therapy.

作者信息

Lee Sang-Hoon, Lee Yoonsuk, Choi Eung Ho

机构信息

Department of Dermatology, Yonsei University Wonju College of Medicine, Wonju, Korea.

Department of Emergency Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea.

出版信息

Ann Dermatol. 2023 May;35(Suppl 1):S59-S62. doi: 10.5021/ad.20.330.

Abstract

Livedoid vasculopathy (LV) is a chronic coagulation disorder characterized by recurrent, painful ulcers on the lower extremities. Methylene tetrahydrofolate reductase () gene polymorphism is associated with coagulopathy. Therapeutic options usually include anti-inflammatory or immunosuppressive agents. However, the condition is still highly challenging to manage and no consensus over the first-line treatment for LV exists. Furthermore, when LV is accompanied with gene polymorphism, clinical presentations could be more severe and resistant to treatment. We report a case of refractory LV accompanied by gene polymorphism, which was successfully treated with hyperbaric oxygen therapy (HBOT). A 63-year-old female patient presented with multiple painful ulcers, atrophie blanches, and retiform purpura on both lower legs and feet. Histopathologic findings were compatible with LV. LV was diagnosed based on these clinicopathological findings. Following the diagnosis, we treated the patient with pentoxifylline, aspirin, systemic corticosteroid, antihistamine, and antibiotics. In spite of six-month treatment, the skin lesions did not improve; hence, HBOT was performed. It was performed at 2.0 absolute atmosphere for 120 minutes each time, three times a week. After 4 sessions, the ulcers began to heal and after 13 sessions, the skin lesions almost healed. During the eight-month follow-up period, the skin ulcers did not recur and the symptoms remained stable. Additionally, it was confirmed that she had gene polymorphism after a genetic test. In conclusion, we wish to provide evidence regarding the effectiveness of HBOT and suggest that HBOT might be a considerable treatment option in refractory LV.

摘要

萎缩性血管病变(LV)是一种慢性凝血障碍,其特征为下肢反复出现疼痛性溃疡。亚甲基四氢叶酸还原酶()基因多态性与凝血病有关。治疗选择通常包括抗炎或免疫抑制剂。然而,这种疾病的管理仍然极具挑战性,对于LV的一线治疗尚无共识。此外,当LV伴有基因多态性时,临床表现可能更严重且对治疗耐药。我们报告一例伴有基因多态性的难治性LV病例,该病例通过高压氧治疗(HBOT)成功治愈。一名63岁女性患者双小腿和足部出现多处疼痛性溃疡、白色萎缩及网状紫癜。组织病理学检查结果与LV相符。根据这些临床病理表现诊断为LV。诊断后,我们用己酮可可碱、阿司匹林、全身用糖皮质激素、抗组胺药及抗生素治疗该患者。尽管治疗了6个月,皮肤病变仍未改善;因此,进行了HBOT。治疗在绝对气压2.0下进行,每次120分钟,每周3次。4次治疗后溃疡开始愈合,13次治疗后皮肤病变几乎愈合。在8个月的随访期内,皮肤溃疡未复发,症状保持稳定。此外,基因检测证实她存在基因多态性。总之,我们希望提供关于HBOT有效性的确切证据,并表明HBOT可能是难治性LV的一种重要治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6bc6/10608380/cd388eb04426/ad-35-S59-g001.jpg

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