成人皮肤型朗格汉斯细胞组织细胞增生症

A case report of adult cutaneous Langerhans cell histiocytosis

报告1例成人皮肤型朗格汉斯细胞组织细胞增生症。患者女,23岁。四肢、头部及面部大量丘疹、结节,无自觉症状2年。皮损组织病理检查示真皮全层片状、条索状单一核细胞浸润,免疫组化示肿瘤细胞CD1a(+)、Langerin(部分+)及Ki-67(约20%+)。透射电镜下肿瘤细胞内见不典型Birbeck颗粒。诊断:皮肤型朗格汉斯细胞组织细胞增生症。鉴于患者一般情况好,无其他器官累及,未予治疗,随访3个月,部分皮损变平及消退。

A case of adult cutaneous Langerhans cell histiocytosis is reported. A 23-year--old female presented with numerous asymptomatic papules and nodules on her limbs and face for about two years. Histopathological examination showed many mononu- clear ceils infiltrated in the dermis, which arranged in sheets or in cords. The immunohistochemical staining revealed the cells stained positive for CDla, Langerin（partial） and Ki-67（about 20%）. Atypical Birbeck granules were found by electronmicroscopy. The diagnosis of cutaneous Langerhans cell histiocytosis was made. Based on the patient＇s good condition and no systemic involve- ment, no treatment was given, The patient was followed up for 3 months, part of the lesions improved and disappeared.