成人朗格汉斯细胞组织细胞增生症1例

A Case of Adult Langerhans Cell Histiocytosis

患者男,48岁,肛周皮肤增生性斑块半年。皮肤科情况:肛周见裙边样增生性斑块,边缘游离,表面光滑。皮损组织病理示:表皮基本正常,真皮乳头水肿,可见大量浆细胞浸润,中下层可见大量增生单个核细胞成结节状浸润,胞质丰富淡染,核椭圆形或肾形,部分可见核沟,并可见大量的嗜酸性粒细胞及淋巴细胞浸润。免疫组织化学示:CD1a(+)、S-100(+)、CD68(+),Ki-67(40%+)。诊断:朗格汉斯细胞组织细胞增生症。治疗:给予口服沙利度胺50 mg,2次/d治疗,3个月后肛周皮损明显消退。

A 48-year-old male patient received skirt-like proliferative plaque with free edges and smooth surface on perianal for half a year.Histopathology showed the epidermis was basically normal,with dermal papillary edema,and a large number of plasma cell infiltration could be seen.A large number of proliferative mononuclear cells in the middle and lower layers were seen as nodular infiltration,rich and light staining of cytoplasm,oval or renal nuclei,some nuclear groove,and a large number of eosinophilic granulocytes and lymphocytes could be seen.Immunohistochemistry showed CD1a(+),S-100(+),CD68(+),Ki-67(40%+).The diagnosis of Langerhans cell histiocytosis was made.After the treatment with oral thalidomide 50 mg twice a day 3 months,the perianal lesions significantly resolved.