成人皮肤朗格汉斯细胞组织细胞增生症1例

目的探讨成人皮肤朗格汉斯细胞组织细胞增生症(LCH)的临床、组织病理学、免疫表型及电镜特征。方法报告1例罕见的成人皮肤朗格汉斯细胞组织细胞增生症,并结合文献分析其临床病理特征。结果该例先后经过4次病理活检,组织学特点为真皮全层弥漫分布的朗格汉斯细胞。瘤细胞表达CD1a、S-100、部分表达Langerin。电镜检查发现Birbeck颗粒。结论成人皮肤弥漫性LCH罕见,误诊率高。确诊应结合组织学特征、免疫表型及超微结构检查综合分析,疾病的预后与发病年龄、受累器官的多少有关。

A Case of Adult Cutaneous Langerhans Cell Histiocytosis

Objective To investigate the clinicopathological,immunophenotypical and electron microscopical features of cutaneous langerhans cell histiocytosis(LCH). Methods A case of rare adult cutaneous LCH was reported and the clinicopathological features were summarized by literature review. Results Skin biopsies were performed four times. Histopathologically,the langerhans cells infiltrated in the dermis diffusely. The tumor cells were positivity for CD1a and S-100,some of them for Langerin immunohistochemically. Birbeck granules were found by electron microscopy. Conclusion Disseminated adult cutaneous LCH is rare and easy to be misdiagnosed. The confirmed diagnosis is based on the aggregate analysis of its histological features,immunophenotype and ultramicrostructure. The prognosis is related with the onset age,the quantity of affected organs.