Mazoyer Elisabeth, Enjolras Odile, Bisdorff Annouk, Perdu Jérome, Wassef Michel, Drouet Ludovic
Consultation Multidisciplinaire des Angiomes, Hôpital Lariboisière, Assistance Publique-Hopitaux de Paris, Université Paris, Paris, France.
Arch Dermatol. 2008 Jul;144(7):861-7. doi: 10.1001/archderm.144.7.861.
To investigate the clinical characteristics of venous malformation of the limbs and trunk and known but poorly appraised associated coagulation disorders. Venous malformations are ubiquitous, slow-flow vascular anomalies known to be occasionally painful because of thrombotic episodes inside the lesion.
Large case series, with screening of accepted standard coagulation tests.
Ambulatory multidisciplinary clinics for vascular anomalies.
This 2-year study (2003-2005) included 118 patients with clinical, radiological, and biological features informative for better defining venous malformation and associated coagulation abnormalities.
The primary outcome was coagulation disorders associated with VM. Secondary measures include anatomic location, extent of lesion, localized pain, and impaired motion.
The mean age of patients was 27 years, and there was a female preponderance of 64%. The venous malformation involved the upper extremity, lower extremity, and trunk in 30%, 58%, and 36% of patients, respectively; it was plurifocal in 22%. Intralesional pain (in 92% of patients) had a higher frequency in female (63%) than in male (47%) patients. Tissular involvement concerned the skin (65%), muscle (73%), bone (13%), joints (12%), and viscera (9%). According to our severity scoring system, cases of less gravity had a score of 2 or 3 (52%), cases of intermediate severity had a score of 4 or 5 (32%), and cases of major severity had a score of 6 to 9 (10%). The most frequent blood coagulation abnormality was a high plasma D-dimer level (> 0.5 microg/mL) (58% of patients), which was correlated with muscle involvement and high severity score and was more frequent in women. The factor VIII-von Willebrand factor complex was documented in 84 patients, and plasma von Willebrand factor level was decreased (<60%) in 23 (27%) of them; 10 of the 84 patients (12%) had more notably decreased levels (<50%).
This study of a large case series of patients with pure venous malformation in the limbs and/or trunk highlights muscle involvement and frequency of pain. It validates that coagulation disorders, present in 58% of our patients, create thrombotic painful events. Under certain circumstances, these disorders entail a risk of hemorrhage because of the progression of localized intravascular coagulopathy to disseminated intravascular coagulopathy.
研究四肢和躯干静脉畸形的临床特征以及已知但评估不足的相关凝血障碍。静脉畸形普遍存在，是一种低流速血管异常，已知因其病变内血栓形成发作而偶尔引起疼痛。
大型病例系列研究，采用公认的标准凝血试验进行筛查。
血管异常门诊多学科诊所。
这项为期2年的研究（2003 - 2005年）纳入了118例具有临床、放射学和生物学特征的患者，这些特征有助于更好地界定静脉畸形及相关凝血异常。
主要观察指标是与静脉畸形相关的凝血障碍。次要指标包括解剖位置、病变范围、局部疼痛和活动受限。
患者的平均年龄为27岁，女性占优势，比例为64%。静脉畸形累及上肢、下肢和躯干的患者分别占30%、58%和36%；22%为多病灶。病变内疼痛（92%的患者有此症状）在女性患者（63%）中的发生率高于男性患者（47%）。组织受累涉及皮肤（65%）、肌肉（73%）、骨骼（13%）、关节（12%）和内脏（9%）。根据我们的严重程度评分系统，轻度病例评分为2或3分（52%），中度病例评分为4或5分（32%），重度病例评分为6至9分（10%）。最常见的血液凝固异常是血浆D - 二聚体水平升高（>0.5微克/毫升）（58%的患者），这与肌肉受累和高严重程度评分相关，且在女性中更常见。84例患者检测了因子VIII - 血管性血友病因子复合物，其中23例（27%）的血浆血管性血友病因子水平降低（<60%）；84例患者中有10例（12%）水平显著降低（<50%）。
这项对大量四肢和/或躯干单纯性静脉畸形患者的病例系列研究突出了肌肉受累情况和疼痛发生率。它证实了58%的患者存在凝血障碍，会引发血栓形成性疼痛事件。在某些情况下，由于局部血管内凝血进展为弥散性血管内凝血，这些障碍会带来出血风险。
