泛发性多核细胞血管组织细胞瘤

Generalized multinucleate cell angiohistiocytoma

报告国内首例泛发性多核细胞血管组织细胞瘤。患者男,37岁。因躯干和四肢丘疹、结节进行性增多就诊。皮损组织病理检查示真皮血管增生,间质散在组织细胞和异常核的多核巨细胞浸润;免疫组化染色:多核巨细胞、组织细胞CD68、ⅩⅢ因子阳性,血管内皮细胞FⅧ相关因子、CD34阳性,S-100蛋白、CD1a阴性。该病是ⅩⅢ因子阳性的树突状细胞增生性疾病病谱中的一种,是一种反应性疾病。

The first case of generalized muhinueleate cell angiohistiocytoma is reported in China. The 37-year-old man presented with multiple papules and nodules on the trunk anti extremities and the lesion increased progressly. The characterized histological feature was dermal vascular proliferation, lymphohistiocytic infiltrate and bizarre multinueleate cells. The resuhs of immunohistochemical stain were thai multinucleate cells were labeled with CD68 and antifactor XⅢa antibody, negalive for S-100 protein anti CD1a, CD34 were positive in the blood vessels. The entity belongs to the spectrum of disease with factor XⅢa^＋ dendrocyte proliferations and was a reactive disease.