Brat H G, Hamoir X, Matthijs P, Lambin P, Van Campenhoudt M
Department of Radiology, St. Elizabeth Hospital, Avenue de Fré 206, B-1180 Brussels, Belgium.
Eur Radiol. 1999;9(1):159-62. doi: 10.1007/s003300050648.
A 40-year-old white man with a 3-year history of mild to severe right thigh and knee pain was referred for radiographic investigation. Radiographs show a fusiform, bilaterally symmetrical enlargement of the diaphyses and metaphyses of the long bones (femur, tibia, fibula, radius and ulna). A narrowed medullary cavity is illustrated on CT scan of the femur. All bones show periosteal and endosteal bone formation. There is no history of familial involvement, trauma, infection or systemic illness. Blood chemistry could not point out any abnormality. Radiographic findings and clinical history suggest the diagnosis of Camurati-Engelmann disease, also known as progressive diaphyseal dysplasia (PDD). This case is of interest because of its rare metaphyseal involvement, mild form and sporadic presentation.
