Osteopoikilosis, also called "osteopecilia" or "spotted bone disease," is a rare condition of bone, manifest by multiple small islands of dense bone formation in areas where normally cancellous bone is found. Ritvo1 states that these lesions "vary in size from 2-3 mm. to 3 cm. occurring frequently in the epiphysis and adjacent parts of the metaphysis, usually being more numerous in the latter. The areas of density lie in the cancellous bone, and, as a rule, are entirely free from the cortex. Patients have been observed over a number of years with no apparent change in the roentgen manifestations." While the apparently hereditary, and essentially benign, course of the disease is stressed in most reports,2-6 a considerable amount of literature has accumulated concerning associated conditions.7-11 Two types, distinguished roentgenologically, are described: linear and millet-like.
Most of these associated defects are related to skeletal and cutaneous lesions. The