Progressive Backbone Dysplasia

Progressive diaphysial dysplasia (PDD) is also called proliferative periostitis, symmetric sclerosing osteodystrophy, Engelmann disease or Camurati-Engelmann disease. The disease was reported by Camurati (1922) and Engelmann (1927) and was named autosomal dominant hereditary bone disease. The disease is characterized by systemic symmetric bone dysplasia, which is manifested by abnormal hyperplasia of the inner and outer periosteum of the long tube, leading to thickening of the cortex, thickening of the backbone and narrowing of the medullary cavity. On the basis of sclerosis, a decrease in plaque bone density is seen Area. The epiphysis is usually normal, but it can also be affected. Therefore, some people claim to call PDD a progressive diaphyseal and epiphyseal dysplasia. Long bone involvement can cause movement disorders and bone pain in patients. Sclerosis of the skull can cause hearing loss, smell loss or loss.