着色性干皮病伴发血管内皮细胞瘤1例

患儿女，9岁，全身皮肤色素斑8年余伴肿物3个月。检查：面部，颈部，胸背上部曝光部位及四肢皮肢欢迎光临性色素沉着斑，颜色呈暗棕色及黑色，深浅不一，间杂白色萎缩性斑点，鼻部，背部，前臂可见大小不等疣状赘生物，触之易出血，背部赘生物作组织病理检查示：真皮内毛细血管呈片状和结节状增生，排列紧密，内皮细胸增生，聚集成实性束或团块，部分呈短俊形，有轻度异形性，可见少量核分裂像，部分区域血管扩张，形状不规则，管壁较薄，内皮细胞增生不明显，管周可见稀疏淋巴细胞，免疫组化染色示：CD31呈灶性阳性（ ），CD34阳性（ ）。诊断：着色性上皮病伴发血管内皮细胞瘤。

One case report of xeroderma pigmentosum accompanied with hemangioendothelioma

A 9-year-old female had pigmented spots on her skin for more than eight years and tumor for three years. Physical examination showed that the pigmented spots were diffusely situated on her skin where was exposed to light, such as face, neck, upper parts of chest and back, and limbs. The color of lesions varied from brown to black. The brown verruciform neoplasms with different size were found on her nose, back and forearms, which were easy bleeding. The skin biopsy revealed that the tumor consisted of short strands, cords, or lump of epithelioid endothelial cells with a few of nuclear atypia and mitotic figures, which exhibited immunoreactivity for endothelial cell markers (CD31 and CD34). Finally this patient was diagnosed as xeroderma pigmentosum accompanied with hemangioendothelioma.