Monomelic melorheostosis of the hand

: Melorheostosis is a rare sclerosing bone dysplasia affecting children and adults. Concerning the etiology it seems to be a metameric abnormality of the tissue of mesodermal origin, which is initiated early in embryonic life prior to the formation of the extremity buds. The flowing hyperostosis is usually associated with limb deformity, soft tissue contractures, severe pain and stiffness of joints. Bone scanning complements standard radiologic studies and permits evaluation of the extent and activity of the disease. Conservative treatment is disappointing. Thus we recommend the excision of the exostoses at an early state to prevent further deformity as is shown in a clinical case report.