Richards Kristen A, Paller Amy S
Department of Dermatology, Children's Memorial Hospital, Northwestern University Medical School, Chicago, Illinois, USA.
Pediatr Dermatol. 2003 Mar-Apr;20(2):124-7. doi: 10.1046/j.1525-1470.2003.20205.x.
Moyamoya disease is a rare, chronic cerebrovascular occlusive disease of unknown etiology. It is characterized by progressive stenosis of the arteries of the circle of Willis leading to an abnormal capillary network and resultant ischemic strokes or cerebral hemorrhages. The association of moyamoya disease with livedo reticularis has been described in a previously reported patient with a factor V Leiden mutation, leading to hypercoagulation. We describe a girl with livedo reticularis and moyamoya disease with extensive cardiovascular malformations, but without a primary coagulopathy.
烟雾病是一种病因不明的罕见慢性脑血管闭塞性疾病。其特征是 Willis 环动脉进行性狭窄,导致异常毛细血管网形成,并引发缺血性中风或脑出血。先前有报道称一名患有因子 V Leiden 突变导致高凝状态的患者,其烟雾病与网状青斑有关。我们描述了一名患有网状青斑和烟雾病且伴有广泛心血管畸形,但无原发性凝血障碍的女孩。