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例1为59岁女性,患糖尿病、尿崩症,经Minirin治疗8年,发现肛周肉芽肿性溃疡和浸润性丘疹3年,乳房下可见少量丘疹。组织学检查示真皮内弥漫性淋巴细胞、嗜酸粒细胞和大组织细胞浸润。免疫病理示组织细胞S100和CDla染色阳性。电镜示浸润细胞中20％含有Birbeck颗粒,证实本病诊断。临床检查无阳性发现。胸片、胸部CT扫描、骨穿均示正常。促性腺激素和促肾上腺皮质激素、生长激素水平较
Example 1 A 59-year-old woman with diabetes mellitus and diabetes insipidus, who had been treated with Minirin for 8 years, had perianal granulomatous ulcers and infiltrative papules for 3 years and had a small papule under the breast. Histological examination revealed diffuse infiltration of intraepithelial lymphocytes, eosinophils and macrophages. Immunohistochemistry showed that the positive staining cells of S100 and CDla. Electron microscopy showed infiltration of cells containing 20% of Birbeck particles, confirmed the diagnosis of the disease. No positive clinical examination found. Chest radiograph, chest CT scan, bone wear are normal. Gonadotropin and adrenocorticotropic hormone, growth hormone levels