Dickson G R, Hamilton A, Hayes D, Carr K E, Davis R, Mollan R A
Department of Anatomy, Medical Biology Centre, Queen's University of Belfast, N. Ireland.
Bone. 1990;11(3):205-10. doi: 10.1016/8756-3282(90)90215-k.
Vanishing bone disease is a rare condition producing local deformity and instability. Fibrovascular tissue replaces bone completely but the mechanism of bone destruction and resorption is unknown and there is controversy regarding the presence or absence of osteoclasts in the disease. Radiography, clinical chemistry, light microscopy, transmission electron microscopy (TEM) and cytochemistry were used to investigate the condition of a young woman presenting early in the disease process. We detected atypical ultrastructure in osteoblasts and endothelial cells. The rare osteoclasts, numerous mononuclear phagocytes and vascular endothelium found in the condition reacted positively for the enzyme acid phosphatase. Aggressive local excision of diseased tissue and insertion of a free vascularized bone graft at an advanced stage of the disease, accompanied by subsequent radiotherapy for residual disease only were successful in rehabilitating the affected forearm and hand.
骨质消失症是一种罕见的疾病，会导致局部畸形和不稳定。纤维血管组织会完全取代骨骼，但骨骼破坏和吸收的机制尚不清楚，并且对于该疾病中是否存在破骨细胞也存在争议。我们使用放射学、临床化学、光学显微镜、透射电子显微镜（TEM）和细胞化学方法来研究一名处于疾病早期的年轻女性的病情。我们在成骨细胞和内皮细胞中检测到了非典型超微结构。在该病症中发现的罕见破骨细胞、大量单核吞噬细胞和血管内皮对酸性磷酸酶呈阳性反应。在疾病晚期积极局部切除病变组织并植入带血管游离骨移植，仅对残留疾病进行后续放疗，成功修复了受影响的前臂和手部。
