以残毁性多关节炎为主要表现的多中心网状组织细胞增生症1例

A Case of Multicentric Reticulohistiocytosis Presenting with Destructive Polyarthritis

患者女,65岁。鞍鼻,头面部、躯干红色丘疹、结节伴手足关节变形10余年。体检:步态蹒跚,头面部、躯干散在多个紫红色圆形绿豆至黄豆大小的丘疹、结节,质软,界清,表面光滑,部分扁平,后背可见片状分布红色丘疹、紫癜,双手关节萎缩变形,活动受限,双膝关节压痛阳性。背部皮损组织病理示:表皮萎缩,真皮可见单核组织细胞和多核巨细胞,胞浆嗜酸性,呈毛玻璃样。诊断为多中心网状组织细胞增生症,长期予以泼尼松口服治疗。

A 65-year-old female presented with red papules, nodules on the head, face and trunk and destructive pol- yarthritis for 10 years. Physical examination showed scattered purple-red papules and nodules on the head, face and trunk, red papules and purpura on the back, joint deformation with limitation of activity. His- topathological finding showed atrophy of epidermis, multinucleated giant cells with ground glass appearance of the eosinophilic cytoplasm in dermis. She was diagnosed as multicentric reticulohistiocytosis and then treated by prednisone for long time.