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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。
One case reported Langerhans cell histiocytosis with perianal verrucous hyperplasia as the first symptom. Children male, 2 years and 5 months, due to perianal lesions 1 year treatment. Dermatology examination: the scalp, ear and trunk more tips to the rice dark red papules and petechiae; perianal see verrucous hyperplasia, partial erosion. Trunk skin lesions histopathological examination: a large number of infiltration of dermal mononuclear cells, oval or horseshoe nucleus, abundant cytoplasm of light, no obvious pathological mitosis, scattered in lymphocyte infiltration. Immunohistochemistry: CD1a, S-100 protein and Langerin (+). Perianal lesions histopathological examination: epidermal hyperkeratosis, the presence of granular layer, acanthosis, a large number of superficial dermis visible infiltration of mononuclear cells, cytoplasm rich red dye, nuclear oval or kidney-shaped, part of the visible nuclear ditch, and Eosinophils and lymphocytes can be seen infiltration. Immunohistochemistry showed S-100 protein (+), CD1a (+), CD68 small amount (+) and Langerin (+). Diagnosis: Langerhans cell histiocytosis (skin and liver). To JLSG-96 chemotherapy induced a course of treatment, the skin lesions significantly subsided.