未定类细胞组织细胞增生病

Indeterminate cell histiocytosis:a case report

报告1例未定类细胞组织细胞增生病。患者女,30岁。全身出现红色丘疹、结节2年。临床表现为无症状、泛发全身的丘疹和结节,分布于四肢、面部及躯干,皮损直径1～3cm,质地坚实,呈黄红色或红棕色。患者无其他系统疾病。皮损组织病理检查:真皮弥漫性组织细胞增生,免疫组化显示增生细胞具有朗格汉斯细胞的特性:CD1a和S-100蛋白阳性,电镜显示细胞质中无Birbeck颗粒。诊断为未定类细胞组织细胞增生病,给予沙利度胺50mg,每日2次口服,阿维A30mg,每日1次口服,获良好疗效。

A case of indeterminate cell histiocytosis is reported. A 30-year-old woman presented with a two-year history of generalized asymptomatic papules and nodules over her face, trunk and extremities. Physical examination showed firm, painless, non-pruritic, yellowish-red or reddish-brown papules and nodules on the face, trunk and extremities, 1 to 3 cm in diameter. She was otherwise healthy. Histopathology showed diffuse infiltration of proliferated histioeytes in the dermis, without epidermotropism or atypia, Immunohistochemical examination revealed positive staining for CDIa and S-100 in the neoplastic cells. Birbeek granules within the cytoplasm of the neoplastic cells were not shown under electron mieruscopy. After two- month treatment with thalidomide （50 mg, twice daily） and acitretin （30 mg, once daily）, marked improvement was observed.