n SA Journal of Radiology - Camurati-Engelmann disease : pictorial essay

Volume 15, Issue 1
  • ISSN : 1027-202X
  • E-ISSN: 2078-6778



Camurati-Engelmann disease (CED), or progressive diaphyseal dysplasia, is a rare sclerosing dysplasia of which 250 cases have been described in the English literature. The disease affects one in a million people and is autosomal dominant with variable penetrance. It was initially described by Cockayne in 1920; Camurati was the first to suggest its hereditary nature in 1922. A single case of muscular wasting and marked bone involvement was reported by Engelmann in 1929. As the name suggests, there is progressive hyperostosis and predominant involvement of the diaphyses.

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