多中心网状组织细胞增生症三例并文献复习

目的 探讨多中心网状组织细胞增生症的临床特点、诊断及鉴别诊断。方法 分析3例多中心网状组织细胞增生症的临床、X线、组织病理和免疫组织化学特征，并复习有关文献。结果 3例患者均有多发性皮肤丘疹和结节，残毁性关节炎，肌肉症状，X线片关节有侵蚀性变化，部分指骨可有囊样骨缺损；肌酶谱均正常；病理均有细胞质呈毛玻璃样的多核巨细胞；1例伴有肺部结节；2例免疫组织化学染色示CD68阳性；AE1／AE3、S－100和HHF35均阴性。结论 多中心网状组织细胞增生症是一种系统性疾病，临床上常伴有肌肉症状，易误诊为皮肌炎，应注意两者的鉴别。

Multicentric reticulohistiocytosis: 3 case report and literature review

Objective To study the clinical manifestation,diagnosis and differential diagnosis of multicentric reticulohistiocytosis.Methods Three cases of multicentric reticulohistiocytosis were reported.The feature of clinical manifestation,radiography,histopathology and immunohistochemical staining were studied.The literature related to multicentric reticulohistiocytosis was reviewed.Results All of the three cases had widespread cutaneous papules and nodules,destructive arthritis,muscle symptom.Radiography of the hands revealed erosions of joints and part of the finger bones had cyst formation.Enzymes of muscle were normal and ground glass cytoplasm multinuclear giant cells were found in pathology.One case was accompanied by lung nodules.Immunochemical staining in two patients revealed positive reaction for CD68,and negative reaction for AE 1/AE 3,S 100 and HHF 35 .Conclusion Multicentric reticulohistiocytosis is a systematic disease.It is always accompanied by muscle symptom and usually misdiagnosed as dermatomyositis.