伴皮肤损害的老人朗格汉氏细胞组织细胞增多症(附3例报道)

Langerhan's cell histiocytosis with skin lesions in elder

目的:报道3例伴皮肤损害的老人朗格汉氏细胞组织细胞增生症(LCH),临床上曾诊断“红皮病”或“嗜酸细胞增多症”。方法:3例患者皮肤损害相似,都有剧烈瘙痒,伴多部位淋巴结肿大。外周血白细胞明显增高和嗜酸细胞不同程度增高。对3例患者皮肤和淋巴结组织行组织病理、免疫组化检测。结果:淋巴结病理显示淋巴结结构破坏,T细胞区有大量单一核细胞为主的浸润,其中多数是S-100(+)和CD1a(+)的朗格汉氏细胞。皮肤病理显示真皮内与淋巴结中浸润的细胞一致。并且大量CD1a(+)细胞和淋巴样细胞向表皮游走,共同形成Pautrier样微脓疡。结论:根据3例患者的皮肤和淋巴结浸润细胞的免疫学标记,诊断为“伴有皮肤损害的老人LCH”。系统给予皮质类固醇激素和干扰素治疗有效。

Objective：Three cases of Langerhan＇s cell histiocytosis in the elder with skin lesions were reported,who had been misdiagnosed clinically as erythroderma or hypereosinophilia.Methods：Clincal materials：Three cases presented the similar skin lesions,usually associated with intensive itching,lymphadenectasis and peripheral leukocytosis and hypereosinophiles.The tissue specimens from skin and lymphonodes of the three patients were detected by histopathologic,immunohistochemical method.Results：The histopathologic features of lymphonodes showed that the structure were destructed,with a large amouot of monomorphous nuclear ceils infiltratation in the areas of T lymphocytes,most of which were S-100（＋）,and CDla（＋） with the immunohistochemical stain.The histologic features of skin showed the same infihrtive cells in dermis as those in lymphonodes.A large number of CDla （＋） cells and lymphoid cells transmigrated to the epidermis,presenting with Pautrier＇s like microabscess.Concluslon：The diagnosis of three cases reported was Langerhan＇s cell histiocytosis with skin lesions in the elder based on their immunologic markers.Systemic corticosteroids and interferon therapies were effective.