多中心网状组织细胞增生症1例

A case report of multicentric reticulohistiocytosis

患者女,35岁。全身红斑、丘疹、结节伴瘙痒7个月,双手指间关节、腕关节、肘关节肿痛2个月。肌电图示左三角肌、右股二头肌肌源性损害。右前臂皮疹组织病理示:真皮可见大量组织细胞和多核巨细胞,细胞体积大,胞浆丰富、嗜酸性,均质或细颗粒状,呈"毛玻璃"样,瘤细胞Vimentin(+),CD68(+),CD163(+),S-100(-),CD1a(-)。诊断:多中心网状组织细胞增生症。经"甲泼尼龙、MTX、羟氯喹"联合治疗后皮疹和关节症状明显改善。

A 35-year-old female patient presented with erythema, papules, nodules accompanied with itching on her face, trunk and extremities for seven months, with sore pain in her hands＂ interphalangeal joints, wrist joints and elbow joint for two months. The electromyography showed muscle damage in the pa- tient＇s left deltoid and in her right biceps femoris muscle. Histopathological examination showed that there were many histiocytes and multi-nucleated giant cells with ＂ground-glass＂ cytoplasm in the dermis. Immu- nohistochemical staining showed positive reaction of Vimentin, CD68, CD163, and negative reaction of S- 100, CDla. The final diagnosis was multicentric reticulohistiocytosis. After the treatment with ＂ methylpred- nisolone, MTX and hydroxychloroquine＂, the skin lesions and arthritic symptoms were significantly im- proved.