n SA Journal of Radiology - Camurati-Engelmann disease : pictorial essay
|Article Title||Camurati-Engelmann disease : pictorial essay|
|Journal||SA Journal of Radiology|
|Author||Neil Bellew and Georg Wagener|
|Publication Date||Mar 2011|
|Pages||20 - 24|
|Keyword(s)||Stellenbosch University, Steve Biko Academic Hospital and Univerity of Pretoria|
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.
Article metrics loading...