Local Osteodysplasias

Abstract

Osteopoikilosis is a hereditary bone disease that causes increased bone density. However, unlike the diffuse bone density of most other sclerosing bone dysplasias, osteopoikilosis is characterised by multiple discrete round or oval radiodensities (spotted bone disease or “osteopathia condensans disseminata”) (Fig. 74.1a, b). Each lesion is an enostosis, a “bone island”. Hundreds of these spots, about 2–5 mm in size, are clustered in epiphyseal and metaphyseal areas of the skeleton and seem to be the result of focal abnormal bone remodelling. The radiodense lesions are scattered symmetrically throughout the skeleton, a feature that helps distinguish this disorder from metastatic carcinoma. The bone tissue in these spots is lamellar and shows evidence of remodelling. These bone lesions are asymptomatic. Some patients with osteopoikilosis have multiple small dermal or subcutaneous fibromas, and the association with these skin lesions suggests an underlying connective tissue disorder.