骨肥大静脉曲张性痣综合征1例

A Case of Osteohypertrophic Varicose Nevus Syndrome

患者女,50岁,双下肢红斑44年,出现增生性皮疹1年。皮肤科情况:双下肢可见片状红斑,左下肢红斑表面有紫红色角化性丘疹,部分区域破溃,表面有血痂,左足踝部可见簇集紫红色丘疹,表面角化,顶端有灰白色角质物。左下肢软组织增生,较右下肢粗大。下肢血管CT成像示:双下肢软组织脉管畸形可能,左下肢、左足软组织肿胀。踝部及大腿紫红色丘疹组织病理示:表皮角化过度,棘层肥厚,表皮突下延,真皮浅层可见扩张毛细血管,管腔大小和形状不规则,血管内皮细胞无增生,腔内充满红细胞。诊断:骨肥大静脉曲张性痣综合征,血管角皮瘤。

A 50-year-old female presented red rash on her legs for 44 years and hyperplastic rash for 1 year.Physical examination showed flaky erythema on both lower limbs,purplish papules arised on the surface of red rash of left limb,some areas were ruptured with blood scab.Clusters of purplish red hyperkeratosis papules covered with pale keratin can be seen on the left ankle.Soft tissue overgrowth let the left lower limb thicker than the right limb.CT angiography of lower limb showed vascular malformation and soft tissue enhanced.Histopathological features of skin lesions took from ankle and thigh were similar and consisted of numerous dilated and congested irregular capillaries in the papillary dermis with overlaying acanthosis and hyperkeratosis,and no proliferation of vascular endothelial cells were seen.Diagnosis of osteohypertrophic varicose nevus syndrome with angiokeratoma was made.