皮肤骨膜肥厚症(附7例报道并结合文献分析)

Pachydermoperiostosis (Report 7 Cases and Analyze Combined Literature)

目的 探讨皮肤骨膜肥厚症临床和X线表现。方法 7例病人经临床及化验检查 ,胫腓骨、尺桡骨、双手双足对称摄片 ,胸透。 3例摄胸片 ,3例作彩色多普勒检查 ,2例摄头颅平片。结果 临床表现为四肢粗大浮肿、杵状指 (趾 ) ,额部皮肤皱纹脑回状加深伴油汗 ,并显示症状和体征不一致。X线特征 :①四肢骨多发对称骨膜增生 ,以胫腓骨、尺桡骨为主 ;于骨干处呈浅淡层状骨膜 ,而骨端处呈花边状 ,密度较高。②四肢骨干、掌骨和跖骨增粗 ,但各关节、第一掌骨和末节指趾骨结构正常。结论 皮肤骨膜肥厚症诊断依靠临床和X线检查 ,须与肢端肥大症、肺性骨关节病及进行性骨干营养不良等鉴别。

Objective To discuss clinic and X ray features of pachydermoperiostosis. Methods Test and radiography were performed in seven patients. Radiographic position included tibia and fibula,ulna and rndius, both of hand and foot, chest and head. Rusults Clinic finding consists of progressive enlargement of the joints and distal extremities, clubbing, coarse facial features and hyperhidrosis. It displayed tiny sympotoms but obvious signs. X ray features included comose symmetry periosteum in limb, skeleton great, normal marrow and joint. Periosteum in diaphses showed layer form and lower density, but in extremitas displayed laced form and higher density. The structure of first metacarpal and end segmental bone are normal. Conclusion The diagnose depended on feature of clinic and radiology and differentiated megalakria, osteopulmonary arthropathy and idiopathic diaphysial atrophy.