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成人Hand-Schuller-Christian综合征1例

Hand-Schuller-Christian Disease in an Adult: A Case Report and Review of the Literature
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摘要 患者女,64岁。全身皮肤红斑和丘疹3年,糜烂、渗液和结痂20d,患"尿崩症"5年。皮肤科情况:双侧眼球外突,胸、腹及背部皮肤见片状红斑,其上分布粟粒至黄豆大丘疹,并见油腻性痂皮,双侧腋下、双乳房下方、腹股沟及会阴部等皱褶部位可见大小不同及形态不规则的糜烂面,表面有浆液性和血性液渗出。皮损组织病理示:角化过度、浅表溃疡、结痂,真皮浅层致密苔藓样混合性炎细胞浸润;免疫组化:皮损内浸润细胞CD1a(+),S100(+)。电镜下细胞浆内可见呈网球拍样的Birbeck颗粒。诊断:Hand-Schuller-Christian综合征。 A 64-year-old female presented with a 3-year-history of generalized skin rashes. The lesions expanded and became crusted and erosive witnin 20 days. She has 5 years history of iabetes insipidus. Haematoxylin and eosin staining showed an extensive epidermotropic and folliculotropic, lichenoid infihration of atypical mono- nuclear cells in the upper dermis. Immunohistochemistry showed that S100, CD1 a were positive and the Bir- beck granules can be found by the electron microscopy. Based on the above findings, the diagnosis was Hand - Schuller - Christian syndrome ( HSC ).
出处 《中国皮肤性病学杂志》 CAS 北大核心 2013年第8期828-830,共3页 The Chinese Journal of Dermatovenereology
关键词 组织细胞增生症 朗格汉斯细胞组织细胞增生症 Hand-Schuller-Christian综合征 Histiocytosis Langerhans cell histriocytosis Hand-Schuller-Christian syndrome
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参考文献8

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