Camurati-Engelmann disease
Progressive diaphysial dysplasia (PDD) is also known as proliferative periostitis, symmetrical sclerosing pachyostosis, Engelmann disease or Camurati-Engelmann disease. The disease is named after Camurati (1922) and Engelmann (1927) reported respectively, and is an autosomal dominant bone disease. The disease is characterized by systemic symmetrical bone development abnormalities, manifested as abnormal proliferation of the inner and outer periosteum of long bones, resulting in thickening of the bone cortex, thickening of the bone shaft and narrowing of the medullary cavity. On the basis of bone sclerosis, patchy areas of reduced bone density can be seen. The epiphysis is generally normal, but it can also be affected, so some people advocate calling PDD progressive diaphysis-epiphyseal dysplasia. Involvement of long bones can cause movement disorders and bone pain in patients. Skull sclerosis can lead to hearing loss and smell loss or loss.