近端指间关节周围胶原沉积症5例并文献复习

Pachydermodactyly:five cases report and review of the literature

目的探讨近端指间关节周围胶原沉积症(PDD)的临床特点,提高对该疾病的认识。方法报道该院5例患者的临床资料并回顾分析国内外相关文献。结果结合该院5例,国内外共报道PDD165例。国内报道的71例患者中,15例(21.1%)有反复挤压关节的病史,4例(5.6%)有心理或精神症状,3例(4.2%)行组织学活检,18例(25.4%)曾被误诊为类风湿关节炎;国外文献报道的94例患者中,34例(36.2%)有关节弹响等皮肤机械损伤,20例(21.3%)有心理或精神症状,74例(78 7%)行组织学检查。结论 PDD预后良好,反复挤压关节及心理或精神因素可能在其中起到一定作用,组织活检有助于诊断,只有充分认识该病才能减少误诊。

[ Objective ] To study the clinical features of pachydermodactyly （PDD） and improve the rheumatologists＇ recognition of the disease. [ Methods ] Five cases of PDD were reported and the world literatmes were reviewed. [ Results ] Totally 165 PDD patients were reported in the world. 15 patients （21.1%） had the evidence of mechanical injury of the fingers, 4 patients （5.6%） bad the psychiatric or neurologic symptoms, and 3 patients （4.2%） were taken the histologic examination in domestic literature. 18 Chinese patients （25.4%） were once misdiagnosed as rheumatoid arthritis. 34 patients （36.2%） presented with the history of mechanical friction, and 20 patients （21.3%） experienced emotional distress in the available literature abroad. 74 patients （78.7%） were taken the histologic exam- ination in other countries. [ Conclusion ] PDD is a benign course. Mechanical injury of the skin and psychiatric or neurologie factors may play a role in the progress of the disease, biopsy may be helpful in diagnosis. Our clinical recognition of PDD would reduce the misdiagnosis of the disease and avoid inappropriate treatment.