多中心网状组织细胞增生症1例  

Multicentric reticulohistiocytosis:A case report

作　　者：翟莉[1] 邱楠 宋惠 ZHAI Li;QIU Nan;SONG Hui(Department of Rheumatology and Immunology, The 960th Hospital of the PLA Joint Logistics Support Force, Tai’an 271000, Shandong, China;Department of Traditional Chinese Medicine, Taian City Central Hospital, Tai’an 271000, Shandong, China)

机构地区：[1]中国人民解放军联勤保障部队第960医院,山东泰安271000 [2]泰安市中心医院中医科,山东泰安271000

出　　处：《北京大学学报（医学版）》2021年第6期1183-1187,共5页Journal of Peking University:Health Sciences

摘　　要：多中心网状组织细胞增生症(multicentric reticulohistiocytosis,MRH)是一种罕见的多系统受累疾病,其病因不明,可能与自身免疫、肿瘤、感染、炎症反应等多因素相关[1],好发于40~50岁的女性[2-3]。MRH的主要临床表现为皮肤黏膜的丘疹、结节和破坏性关节炎[4]。本文报道1例误诊为类风湿关节炎的MRH,以期提高对MRH的认识。A 65-year-old woman developed erythema,papules and nodules over the body.Some nodules of her auricles and hands like string beads.Besides,she suffered from symmetrical swelling and pain of multiple joints,morning stiffness with deformity of joints;She had elevated erythrocyte sedimentation rate and C reactive protein levels;Her rheumatoid factor and antinuclear antibody were positive;Joints destruction was found with X-ray imaging;Skin pathology showed Dermal infiltrate of abundant histiocytes,part of them with a ground-glass appearance;A CD68 immunohistochemical stain was positive and the cells were negative for S100,CD1a.These findings were diagnostic evidences of multicentric reticulohistiocytosis(MRH).The patient received high-dose of glucocorticoids combinated with immunosuppressive agents,and achieved a satisfactory effect.MRH was a rare multisystem disease characterized by papulonodular mucocutaneous and destructive arthritis,and its pathogeny was not yet completely understood.The typical lesions of MRH were hard papules or nodules that usually occured on the hands,face and arms.Classic coral bead appearance from periungual cutaneous nodules that were characteristic of MRH.MRH was an inflammatory joint disease,affecting almost all the appendicular joints and characterized by joint multiple,symmetrical,destructive,progressive disability.Joints destruction of the distal interphalangeal joints was a unique feature of MRH.In addition to skin and joints,it could also involve other systems.There were no diagnostic laboratory markers for MRH.Laboratory examinations had often been found to be non-specific.Imageological examination mainly showed bone and joint destruction.Skin biopsy was the best test to diagnose MRH,the typical histopathological findings included an infiltrate with histiocytes and multinucleated giant cells with a ground-glass appearing in eosinophilic cytoplasm,and the immunohistochemical stain was positive for CD68.The diagnosis was typically made based on the clinical presentation,supportive ra

关 键 词：多中心网状组织细胞增生症 诊断 治疗 

分 类 号：R59[医药卫生—内科学]