以肛周疣状增生物为首发症状的朗格汉斯细胞组织细胞增生症

Perianal vegetation as the first manifestation of Langerhans cell histiocytosis:a case report

报告1例以肛周疣状增生物为首发症状的朗格汉斯细胞组织细胞增生症。患儿男,2岁5个月,因肛周皮损1年就诊。皮肤科检查:头皮、耳后及躯干较多针尖至米粒大暗红色丘疹及瘀点;肛周见疣状增生物,部分糜烂。躯干皮损组织病理检查:真皮大量单个核细胞浸润,胞核椭圆或马蹄形,胞质丰富淡染,未见明显病理核分裂象,散在淋巴细胞浸润。免疫组化:CD1a、S-100蛋白及Langerin均(+)。肛周皮损组织病理检查:表皮角化过度,颗粒层存在,棘层肥厚,真皮浅层可见大量单个核细胞浸润,胞质丰富红染,核椭圆形或肾形,部分可见核沟,并可见嗜酸性粒细胞及淋巴细胞浸润。免疫组化示S-100蛋白(+)、CD1a(+)、CD68少量(+)及Langerin(+)。诊断:朗格汉斯细胞组织细胞增生症(皮肤和肝脏)。予JLSG-96方案诱导化疗一个疗程后,皮损明显消退。

A case of Langerhans cell histioeytosis with perianal vegetation as the first symptom is reported. A 29-monthold boy presented with perianal lesions for 1 year. Physical examination showed many dark red papules and petechiae on the scalp, ears and trunk. Perianal vegetation with partial erosion was seen. Histopathologieal examination of trunk lesion showed a large number of mononuclear cells in the dermis with oval or horseshoe-shaped nucleus and abundant, lightly stained cytoplasm, without apparent pathological mitotic figures. There was scattered lymphocytic infiltration. Immunohistochemieal staining of trunk lesion showed positive for CDla, S-100 protein and Langerin. Histopathologieal examination of perianal lesion revealed epidermal hyperkeratosis, presence of granular layer, acanthosis, and a large number of mononuclear cells, some eosinophils and lymphocytes in the superficial dermis. The mononuelear cells were rich in red-stained cytoplasm, with oval or kidney-shaped nucleus, and some with nuclear grooves. Irnmunohistoehemistry of perianal lesion showed that the tumor cells were positive for S-100 protein, CDla, CD68 and Langerin. The patient was diagnosed as Langerhans cell histiocytosis （skin and liver）. The skin lesions were markedly subsided after the one course of JLCH96 induction chemotherapy.