Camurati-Engelmann disease: a late and sporadic case with metaphyseal involvement

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 sho w a fusiform, bilaterally symmetrical enlargement of the diaphyses and meta physes of the long bones (femur, tibia, fibula, radius and ulna). A narrowe d medullary cavity is illustrated on CT scan of the femur. All bones show p eriosteal and endosteal bone formation. There is no history of familial inv olvement, trauma, infection or systemic illness. Blood chemistry could not point out any abnormality. Radiographic findings ana clinical history sugge st the diagnosis of Camurati-Engelmann disease, also known as progressive d iaphyseal dysplasia (PDD). This case is of interest because of its rare met aphyseal involvement, mild form and sporadic presentation.