皮肤伴巨大淋巴结病性窦组织细胞增生症临床病理观察

摘要

Objective To study a case of entirely confined to skin with massive lymph ad enopathy sinus histiocytosis, and review of literatures, and to discuss its clinical pathologic features and differential diagnosis. Methods Routine pathological examination, H E staining and im-munohistochemical staining ( S- 100, CD68, Lys, Mac387, CD31 , Cdla, X Ⅲ and CD34 ) and light microscopic observation were carried out in cutaneous lesion of a patient, and its pathological features, immunophenotype and differential diagnosis were studied and analyzed. Results Histologically the lesions were mainly in dermis layer of skin, at low magnification, blue stained area and light pink area were alternately arranged ( important diagnostic tips of background lesions ); in an inflammatory background with fiber cells segmentation; large number of monocytes or mul-tinucleated cells and scattered lymphocytes, plasma cells, neutrophil infiltration, and macrophage tissue cells can be seen in phagocytosis of small lymphocytes. Immunohistochemical examination showed macrophagic tissue cells with S-100 strong( + ), CD68 and Lys( + ), Mac387 ( + ) and CD31( + ), but Cdla, X Ⅲ and CD34 were not expressed. The pathological diagnosis: skin with massive lymp had enopathy sinus histiocytosis. Postoperative follow -up for 5 years, no local recurrence or distant metastasis had been seen. Conclusion Massive lymp had enopathy sinus histiocytosis entirely confined to skin is a rare non - neoplastic disease, its clinical behavior is benign with malignant potential, and it is easily to be misdiagnosed as malignant tumor of lymphoreticular system. Definitive diagnosis mainly depends on its distinctive histopathological features supplemented by immunohistochemical staining.%目的 探讨完全局限于皮肤的伴巨大淋巴结病性窦组织细胞增生症的临床病理特点及鉴别诊断.方法 对1例皮肤病变进行常规病理检查,伊红染色(HE)及免疫组化(S-100,CD68,Lys,Mac387,CD31,CD1a,ⅩⅢa因子和CD34)染色,光镜观察,分析其病理学特征、免疫表型及鉴别诊断.结果 组织学上病变主要在皮肤真皮层,低倍镜下见深蓝染区域与淡粉染区域相间(重要诊断提示性背景病变);在炎症背景中有纤维分割;细胞组成见大量的单核或多核的组织细胞及散在的淋巴细胞、浆细胞、中性粒细胞等浸润,同时巨噬性组织细胞内可见吞噬的小淋巴细胞.免疫组化显示巨噬性组织细胞S-100强(+),CD68和Lys(+),Mac387(+),CD31(+),不表达CD1a、ⅩⅢa因子和CD34.病理诊断:皮肤伴巨大淋巴结病性窦组织细胞增生症.术后随访5年,无局部复发或远处转移.结论 完全局限于皮肤的伴巨大淋巴结病性窦组织细胞增生症是很少见的非肿瘤性疾病,临床行为属于良性/潜在恶性,易被误诊为淋巴网织系统恶性肿瘤.明确诊断主要依靠其独特的组织病理学,并辅以免疫组化标记.