青斑样血管病的治疗进展

青斑样血管病是一种慢性、复发性、疼痛性血管堵塞性疾病,以小腿和踝部周围网状青斑、溃疡、星状象牙白色萎缩性瘢痕为特征。其发病机制未明,目前治疗方法较多,包括抗凝剂、类固醇激素、抗血小板、静脉注射免疫球蛋白等,但尚无统一的有效的治疗方案。     

青斑样血管病64例皮损特点分析

【摘要】 目的 分析青斑样血管病网状青斑和紫癜性皮损的特点、分布规律。方法 回顾中国医学科学院皮肤病医院2017年7月至2020年10月诊治的64例青斑样血管病患者的临床资料。结果 64例中男23例,女41例;年龄13 ~ 54岁,发病年龄7 ~ 51岁,48例25岁前发病;病程6个月至10年。49例夏季发病或病情加重,皮损基本表现为坏死性不规则紫癜、紫癜性皮病样红斑、不规则溃疡、网状青斑、毛细血管扩张、不规则的白色萎缩性瘢痕和色素沉着。64例溃疡和坏死性紫癜多为不规则形,发生于足背和踝关节周围。40例有紫癜性皮病样的皮损表现,其中32例表现为色素性紫癜,8例表现为毛细血管扩张性紫癜。4例出现麻木刺痛等神经末梢受损的症状。结论 青斑样血管病的皮损表现多样,对于出现广泛网状青斑、紫癜样皮病样皮损、麻木等症状的患者,需做好鉴别诊断。     

伴PROS1基因突变的白色萎缩

报告1例伴PROS1基因突变的白色萎缩。患儿女,16岁。反复双足瘀斑及溃疡伴疼痛4年。皮肤科检查:双侧足背及踝周可见片状紫红色斑片、点状坏死、色素沉着及结痂,中央可见象牙白色形状不规则的萎缩性瘢痕。皮损组织病理检查:真皮内小血管增生,管壁增厚伴透明变,管腔内纤维素沉积。基因筛查示PROS1基因突变。诊断:伴PROS1基因突变的白色萎缩。予皮下注射低分子肝素钙及口服华法林等抗凝治疗后,皮损基本痊愈,随访1年未复发。     

白色萎缩1例

患者女,42岁。因双踝部出现瘀斑、萎缩性瘢痕4年,于2004年6月来我所就诊。皮损初发于左踝部,为紫红色斑疹,数日后呈暗紫色,少数皮损破溃,呈粟粒大溃疡,伴疼痛,表面有少量黄色分泌物。溃疡缓慢愈合,遗留白色萎缩性瘢痕和色素沉着,愈后不久原部位又有新发皮损,并向四周蔓延,渐累及右踝部。皮损反复发作。体格检查:系统检查未见异常。皮肤科检查:内、外踝部见4处直径5～8cm暗红斑,边缘不规则,其间布有较密集的针头大紫色瘀点,中心有粟粒大溃疡,表面覆血痂、白色鳞屑,散在形状不规则和星状瓷白色萎缩性瘢痕及色素沉着(图1)。皮损组织病理检查:表…     

节段透明性血管炎1例

临床资料患者男,46岁。主因双下肢红斑、溃疡伴疼痛反复发作10年。10年前无明显诱因双下肢出现米粒至绿豆大红色斑疹,数日后变为暗紫红色,后逐渐扩大,伴疼痛、以胫前、踝部为重。部分皮损自行破溃,形成溃疡,可缓慢愈合,遗留白色萎缩性瘢痕,萎缩性瘢痕上新发皮损。外院按“变应性血管炎”给予雷公藤、复方丹参滴丸、维生素C等治疗,皮损略有缓解,未完全治愈。     

白色萎缩合并未分类结缔组织病1例

报告1例白色萎缩合并未分类结缔组织病.患者女,33岁,双小腿出现许多象牙白色萎缩性瘢痕,伴毛细血管扩张,色素沉着,溃疡.皮肤组织病理检查见真皮浅层小血管内皮细胞增生,少数管腔闭锁,血管壁有纤维蛋白样物质沉积和透明变性,给予扩血管,抗凝,血浆置换治疗后好转.     

姐妹同患白色萎缩

例1女，2l岁，双侧踝部及足背疼痛性红斑及白色瘢痕6年。皮损初发于踝周，为数个米粒至绿豆大的红色斑疹，散在分布，数日后变为暗紫红色，后逐渐扩大至1分硬币大片状斑疹，伴轻微触痛。局部自行破溃形成浅溃疡，溃疡缓慢愈合，遗留白色萎缩性瘢痕及色素沉着，自觉烧灼感，皮损反复发作。     

阿布昔替尼治疗青斑样血管病一例

患者,女,31岁。双下肢红斑、丘疹、溃疡伴疼痛2年余,加重3天。临床结合组织病理诊断为青斑样血管病,常规治疗欠佳,给予患者口服阿布昔替尼100 mg每日1次,治疗15周后,红斑、丘疹消退,萎缩性瘢痕及色素沉着减轻,溃疡愈合,疼痛得到控制。     

白色萎缩一例

患者女，19岁。因双踝部出现瘀斑、萎缩性瘢痕5年，于2006年8月来我院就诊。皮损初发于双侧踝部，为紫红色斑疹，数日后呈暗紫色，少数皮损破溃，呈粟粒大溃疡，伴疼痛。溃疡缓慢愈合，遗留白色萎缩性瘢痕和色素沉着，愈后不久原部位又有新发皮损，并向四周蔓延，皮损反复发作。曾到多家医院就诊，先后诊断为湿疹、皮炎等，给予抗组胺药及糖皮质激素外用，病情无缓解。[第一段]     

青斑样血管病的诊疗进展

青斑样血管病是一种非炎性、真皮内血管阻塞性皮肤病,以双下肢远端反复出现疼痛性溃疡,网状青斑以及瓷白色萎缩性瘢痕为主要临床表现。本病病因及发病机制不明,可能与机体高凝状态,纤维蛋白溶解障碍和/或与免疫系统疾病相关。目前无特效疗法,主要以缓解疼痛、防止皮损进展为目的。     

Livedoid vasculitis responding to PUVA therapy

BACKGROUND: Livedoid vasculitis is a chronic disorder manifested as recurrent, painful, reticulated, and ulcerative lesions of the legs, which result in ivory atrophic scars with peripheral telangiectasia and hyperpigmentation. Its etiology remains obscure and therapy is difficult. In this study, we evaluated the clinical efficacy of psoralen plus UVA (PUVA) therapy and its side-effects in the treatment of livedoid vasculitis. METHODS: Eight South Korean patients with livedoid vasculitis were treated with UVA and 8-methoxypsoralen (8-MOP). Systemic PUVA was started with 4 J/cm2 of UVA two or three times a week, and then the dose was increased by 0.5 or 1 J/cm2 increments at each subsequent treatment as tolerated. The effects of treatment were evaluated using photographs of before, during, and after the study. RESULTS: All patients experienced rapid cessation of new lesion formation, significant symptom relief, and complete healing of primary lesions. The mean times for each of the above were 3.6, 5.9, and 10 weeks, and the mean cumulative doses of UVA for each of the above were 55.9, 96.8, and 197.9 J/cm2, respectively. The patients tolerated PUVA therapy well without unacceptable side-effects. CONCLUSIONS: We propose that systemic PUVA using 8-MOP should be investigated further as an alternative treatment for patients with livedoid vasculitis.     

青斑血管炎的研究进展

青斑血管炎是由于局部皮肤血管阻塞引起的疾病。主要表现为好发于小腿、踝部的红色、紫癜样斑疹、丘疹,形成疼痛剧烈的溃疡,最终遗留瓷白色萎缩瘢痕,称为“白色萎缩”。组织病理学显示病变局部缺乏甚至没有炎症反应,现多认为该病与局部血栓形成及其他自身免疫病相关。治疗多采用抗凝为主的综合治疗。     

Livedoid vasculopathy associated with heterozygous protein C deficiency

Livedoid vasculopathy is characterized by recurrent painful ulceration of the feet, ankles and legs that heals with residual white atrophic scars. For many years, livedoid vasculopathy has been considered to be a primary vasculitic process. Recently, however, there has been a trend towards considering livedoid vasculopathy as an occlusive vasculopathy due to a hypercoagulable state. Livedoid vasculopathy (under the designation livedo vasculitis) was first reported to be associated with protein C deficiency in 1992. We describe an additional patient with livedoid vasculopathy associated with heterozygous protein C deficiency. This second reported case suggests that protein C deficiency may be one cause of the hypercoagulable condition in these patients and demonstrates the necessity for further investigation of thrombogenic factors underlying the disease.     

Livedoid vasculopathy as a marker of systemic disease: report of two cases

The livedoid vasculopathy is an obstructive vascular process of etiology not yet fully known, being possibly associated with several prothrombotic events. It is clinically characterized by the presence of painful and recurring purpuric lesions, which usually suffer ulceration and evolve with formation of white atrophic scars usually located in the lower limbs. Two cases are here reported of painful ulcerated lesions on the lower limbs, in which the identification of VL enabled the diagnosis of systemic diseases.     

23例青斑样血管病临床分析

目的：提高对青斑样血管病的临床认识及诊断能力，探讨有效的治疗方法。方法：回顾性分析2010-2017年诊断的青斑样血管病患者的临床资料，包括临床及组织病理特点、治疗方法及疗效。结果：诊断了23例青斑样血管病患者，其中男6例，女17例，发病年龄(24.86±7.63)岁；皮损主要发生于踝周（21/23），皮损表现为疼痛性紫癜、溃疡及象牙色萎缩；组织病理改变主要表现为真皮浅中层小血管壁纤维素样变性及管腔内透明血栓形成；治疗上以抗凝、抗血小板聚集及促纤溶为主，己酮可可碱、达那唑、阿司匹林治疗效果好。结论：青斑样血管病具有特征性临床及组织病理学改变，但早期容易误诊误治，己酮可可碱有助于本病的治疗及预防复发。     

Warfarin therapy for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia

BACKGROUND: Livedoid vasculopathy is an idiopathic, chronic disorder manifested by painful, purpuric macules on the lower extremities that superficially ulcerate, resulting in atrophic, stellate scars with peripheral telangiectasias and hyperpigmentation. OBSERVATIONS: A 50-year-old man presented with recurrent, painful ulcerations on the medial aspect of his malleoli and calves. The clinical presentation, histologic findings, and results of laboratory evaluation confirmed the diagnosis of livedoid vasculopathy in this case. Despite being refractory to treatment with multiple other medications, the lesions responded dramatically to oral warfarin sodium therapy. CONCLUSION: Treatment with warfarin may be a beneficial therapy for patients with livedoid vasculopathy.     

Case of livedoid vasculopathy with peripheral neuropathy successfully treated with low-dose warfarin

We report herein a case of a 28-year-old woman with persistent livedo racemosa and recurrent ulcerations on the lower extremities. The clinical presentation, together with histopathological findings of vascular occlusion without overt vasculitis in the dermis, led to the diagnosis of livedoid vasculopathy. The patient experienced recurrence of ulcerations and developed peripheral neuropathy affecting the distal extremities during the course of treatment with sarpogrelate hydrochloride. The skin lesions and neurological symptoms improved dramatically after adding low-dose warfarin potassium to the treatment regimen. This case suggests that administration of low-dose warfarin is an effective therapy of choice for patients with livedoid vasculopathy.     

温阳活血利水法对冠心病心衰心功能Ⅲ级以上患者LVEF及BNP的影响

目的评价以温阳活血利水法为组方的中药对冠心病心衰心功能DI级以上患者左心射血分数(LVEF)及脑钠肽(BNP)的影响。方法选取2016年10月-2018年12月辽阳市中医院心内科住院的冠心病心衰、心功能Ⅲ-Ⅳ级患者120例,按照随机数字表法,随机分为治疗组和对照组,每组60例;治疗组给予西医常规治疗加温阳活血利水法组方中药,对照组给予西医常规治疗;疗程28 d观察两组患者治疗前后的中医证候积分、心衰积分、左室射血分数(LVEF)、脑钠肽(BNP)、6min步行距离及患者治疗后12个月的再住院率和病死率。结果治疗后两组患者中医证候积分、心衰积分、LVEF、BNP、6min步行距离均较治疗前显著改善,且治疗组改善更明显,差异有统计学意义(P<0.05);治疗12个月后,治疗组患者的病死率明显低于对照组(P<0.05)。结论在西医规范治疗基础上加用溫阳活血利水法组方中药,可以显著改善心衰患者的心功能,极大提高患者生活质量。     

20例青斑血管炎患者的临床特点和治疗分析

目的 探讨青斑血管炎患者的临床特点和治疗方案.方法 回顾性分析20例青斑血管炎患者的临床资料.结果 青斑血管炎患者以女性发病居多(男:女为5:15),其高发年龄为14～20岁,夏季发病或病情加重,在14例患者中抗心磷脂抗体阳性9例.治疗方案中将抑制免疫反应、抗炎、抑制白细胞趋化、抗凝、促纤溶等药物联合应用.根据患者的病情,将上述药物进行组合,从多个环节阻止疾病的发生和发展,药物的维持和不良反应的检测对疾病治疗也很重要.结论 青斑血管炎可在多环节上多种药物联合治疗,但各种药物的作用机制有待进一步探讨.