Liu Ping-Ping, Shuai Zong-Wen, Lian Li, Wang Kang
Department of Rheumatology and Immunology, Arthritis Research Institute, The First Affiliated Hospital of Anhui Medical University, Hefei 230000, Anhui Province, China.
World J Clin Cases. 2023 Jan 16;11(2):456-463. doi: 10.12998/wjcc.v11.i2.456.
Multicentric reticulohistiocytosis (MRH)/systemic lupus erythematosus (SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopathology, and immunopathology. Here, we report the case of a 30-year-old woman with SLE who developed MRH.
A 30-year-old woman with a history of polyarthritis for the past 12 years had multiple skin nodules on her body for 10 years, including the sacrococcygeal area, dorsum of the hands, interphalangeal joint of the feet and sternoclavicular joint. The histopathology of a biopsy of the distal interphalangeal joint of the hands revealed granulomatous inflammation, fibrous hyperplasia with ground-glass degeneration, inflammatory cell exudation and focal necrosis. The immunohistochemical stains showed positive staining for CD68 and negative staining for S100 and acid-fast staining. The patient was diagnosed with SLE with MRH. Her symptoms were improved after a combined treatment of prednisone, hydroxychloroquine and cyclophosphamide.
MRH/SLE overlap syndrome is difficult to diagnose and treat. Cyclophosphamide may be an alternative choice for the treatment of MRH.
多中心网状组织细胞增多症（MRH）/系统性红斑狼疮（SLE）重叠综合征在临床上是一种罕见疾病，通过特征性临床表现、组织病理学和免疫病理学进行诊断。在此，我们报告一例30岁患SLE并发展为MRH的女性病例。
一名30岁女性，有12年多关节炎病史，身体出现多个皮肤结节10年，包括骶尾部、手背、足趾间关节和胸锁关节。手部远端指间关节活检的组织病理学显示肉芽肿性炎症、伴有玻璃样变性的纤维增生、炎性细胞渗出和局灶性坏死。免疫组化染色显示CD68阳性，S100和抗酸染色阴性。该患者被诊断为SLE合并MRH。经泼尼松、羟氯喹和环磷酰胺联合治疗后症状改善。
MRH/SLE重叠综合征难以诊断和治疗。环磷酰胺可能是治疗MRH的一种替代选择。
