Bied J C, Malsh C, Meunier P
Rev Rhum Mal Osteoartic. 1976 Mar;43(3):193-9.
The authors report two cases of melorheostosis discovered in adults because of fairly intense pain and deformation of the affected limb. The radiological appearances were typical. Isotope bone scintigraphy showed strong hyperfixation in the affected bones. A histomorphometric study of a double iliac bone biopsy showed on the one hand, cortical hypertrophy, normal osteonic architecture, and normal lamellar texture in the pathological regions, and, on the other hand, the dynamic character of the healthy bone close the lesions. In one of the patients an original attempt at treatment with diphosphonate (1 200 mg/day of EHDP for 5 months) resulted in a clear improvement in the bone pain. The disease is considered to be a mesodermic dystrophy. It seems to be due to strong hyperactivity of the subperiosteal bone accretion, which, normally, persists only at a weak level in adults.
作者报告了两例成年期发现的骨肥大症病例，病因是患侧肢体相当剧烈的疼痛和畸形。放射学表现典型。同位素骨闪烁扫描显示受累骨骼有强烈的放射性浓聚。对双侧髂骨活检进行的组织形态计量学研究显示，一方面，病理区域存在皮质肥厚、骨单位结构正常和板层纹理正常，另一方面，病变附近健康骨骼具有动态特征。其中一名患者尝试使用双膦酸盐进行治疗（依替膦酸二钠1200毫克/天，持续5个月），骨痛明显改善。该病被认为是一种中胚层发育不良。似乎是由于骨膜下骨质增生的强烈活动亢进所致，而在正常情况下，这种增生在成年人中仅处于较弱水平。
