骨膜增生厚皮症5例分析

Pachydermoperiostosis:5 Cases Report and a Review of Literature

目的通过探讨骨膜增生厚皮症的临床和X线表现,提高对本病的认识。方法总结5例资料完整的骨膜增生厚皮症患者的临床及X线表现,5例均行胫腓骨、尺桡骨、膝关节、踝关节、肘关节、腕关节、双手、双足对称摄片及胸片检查,3例摄头颅平片。结果临床表现为四肢粗大、杵状指(趾)、皮肤增厚、额部皮肤皱纹脑回状加深伴油汗;X线特征性表现为:四肢骨多发对称骨膜增生,以胫腓骨、尺桡骨为主,大多全段,骨膜增生表现多样,为层状、波浪状或不规则隆起状,四肢骨干增粗,但各关节结构正常。结论骨膜增生厚皮症诊断依靠临床表现和X线检查,须与肺性骨关节病、肢端肥大症及进行性骨干营养不良等鉴别。

Objective To discuss the clinical manifestations and x-ray features of Pachydermoperiostosis. Methods Clinical manifestations and X-ray features of five patients with Pachydermoperiostosis were summarized. All patients take x-ray examination of bilateral tibia, fibula, ulna, radius, knee joint, ankle joint, elbow joint, wrist joint, hand, feet and chest. Three patients took skull x-ray examination. Results Clinical manifestations includes progressive enlargement of limbs, clubbed fingers/toes, thickness of skin, coarse facial features and hyperhidrosis. X-ray features includes symmetric periosteal proliferation in limbs with enlargement of the shaft, which mostly involved the section of tibia, fibula, ulna and radius. The manifestations of periosteal proliferation have variety. The structure of joints are normal. Conclusions The diagnosis of Pachydermoperiostosis depends on clinical and radiological features and should be differentiated from pulmonary osteoarthropathy, acromegaly, osteopubnonary arthropathy and idiopathic diaphysial atrophy.