朗格汉斯组织细胞增生症1例并文献复习

Langerhans Cell Histiocytosis:A Case Report and Review of Literatures

报告1例以皮肤损害就诊的朗格汉斯组织细胞增生症。患儿男,2岁半,因头面、躯干、下肢红斑、丘疹、脓疱一年半入院。皮肤组织病理检查:大量单个核细胞弥漫分布于真皮乳头层并向表皮侵入,单个核细胞体积较大,胞质淡染,核折叠成肾形,中央空泡化,可见线状沟,呈咖啡豆样外观。免疫组织化学法:CD1α及S-100染色阳性。诊断为朗格汉斯组织细胞增生症。同时结合相关文献对朗格汉斯组织细胞增生症的临床表现、诊断和预后等进行分析。

A case of Langerhans cell histiocytosis diagnosed from skin lesions was reported.A two and a half-year-old boy presented with erythema,papules,and pustules on his head,face,truck and legs.The lesions had been presented for one and a half years.Histopathological examination of the skin revealed sheets of mononuclear histiocytes infiltrating in dermis and epidermis.The histiocytes were quite large,and showed pale cytoplasm,and folded nuclei（reniform nuclei）with central vacuolization and prominent nuclear groove.These chraracteristics gave the cells a coffee bean like appearance.Immunohistochemical staining was positive for CD1α and S100.A final diagnosis of Langerhans cell histiocytosis was made.A review of the literatures on clinical features,diagnosis and prognosis of Langerhans cell histiocytosis is also provided.