厚皮指症

目的：总结厚皮指症的临床特点，提高对厚皮指症的认识，减少误诊误治。方法：分析6例患者的临床资料、关节受累情况、实验室检查、X线平片和核磁共振成像、组织病理改变及诊治经过并随访。结果：男性5例，女性1例，平均年龄：16．3±1．8岁。主要表现为双手对称性近端指间（PIP）关节两侧无症状性软组织扁平梭形膨大。实验室检查未发现异常；6例双手X线检查均见受累关节周围软组织肿胀；1例左手核磁共振成像见：近端指间关节周围软组织增厚；1例左手第5PIP关节膨大处软组织活检见：表皮过度角化，真皮层纤维组织增生。院外2例诊断不明，3例误诊为“类风湿关节炎”予中成药或中草药治疗半个月-1个月停药。6例诊断厚皮指症后未治疗，5例随访5-26（13．9±9．2）个月，病情无明显变化。结论：厚皮指症的诊断主要依据特征性临床表现；多因素参与厚皮指症的发生；易误诊甚至误治；该病呈良性经过，无需特殊治疗。

PACHYDERMODACTYLY

Objective：To summarize clinical features of pachydermodactyly and decrease misdiagnosis. Methods：The clinical data of 6 pachydermodactyly cases including laboratory tests, condition of involved joints, manifestation of imaging and histopathology were collected.Results：The mean age of onset was 16.3±1.8 year, 5 male and 1 female. After experienced a delitescence course, the major manifestations of bilateralis non-inflammatory fusiform intumescentiae swelling of proximal interphalangeal （PIP） joints were observed. Abnormal laboratory tests were not found in all patients. X-ray showed soft tissue swelling at involved joints in all patients and MRI showed pachyderma in 1 patients. Epiderm hyperkeratosis and subcuticula fibroplasias were found at PIP 5 joints in 1 patient. Two cases were diagnosed unknown and 3 patients were diagnosed with rheumatoid arthritis and treated with traditional Chinese medicine. After final diagnosis, all of them stopped therapying. No change were found in 13.9±9.2 months follow-up. Conclusion：The diagnosis of pachydermodactyly depends on clinical manifestations.Multiple factors beget the disease. For a well prognosis, pharmacal therapy is not necessary.