摘要目的 分析近端指间关节周围胶原沉积症患者临床特征.方法 总结本院收治4例该病患者临床资料,实验室检查,手关节X线、MRI及肿胀组织的病理活检.结果 4例患者均为青少年,男3例,女1例,发病年龄16～22岁,临床表现为无症状性近端指间关节扁平状肿胀,实验室检查均正常,手X线示软组织肿胀,MRI检查未见滑膜增生及软骨破坏,肿胀组织病理活检示胶原纤维组织沉积.结论 本病为一良性疾病,认识不足易误诊为类风湿关节炎等风湿病,认识此疾病可避免误诊误治.

abstractsObjective To investigate the clinical manifestations of pachydermodactyly. Methods Four patients with pachydermodactyly were enrolled and their clinical presentations and laboratory tests were ana-lyred. Results The mean disease onset age ranged from 16 to 22. Three of them were male,one was female. Clinical presentations included chronic asymptomatic soft tissue swelling of proximal interphalangeal (PIP) joints. No abnormal results were found in laboratory tests. X-ray showed only soft tissue swelling. MRI of the PIP showed no articular damage. And the histopathology showed increased dermal accumulation of collagen fibers. Conclusion Paehydermodactyly is benign. It is important to recognize and diagnose this disease without mis-take it for rheumatoid arthritis or other arthritis involving PIP joints.