[Van Buchem disease. Study of 2 cases and review of the literature]

We report two members of one family, a 51-year-old man a 16-year-old son, with enlargement of the jaw, palatine taurus, endosteal sclerosis of the neurocranium and symmetrical diaphyseal cortical thickening. On the basis of those typical findings, the diagnosis of recessive endosteal hyperostosis (Van Buchem's disease) was mode. This unusual hereditary sclerosing bone dysplasia is discussed with respect to the clinical and radiological features as well as its distinction from other sclerosing disorders.