Paget’s disease of bone was described as “osteitis deformans,” a “chronic inflammation of bone” by Sir James Paget in an address to the Royal Medical Chirurgical Society of London in 1876.

His original description was masterful and thus has withstood the test of time. Paget’s disease of bone describes an abnormal osseous (bony) structure whereby isolated and sometimes contiguous areas of the skeleton undergo changes leading to clinical deformity for some of those affected. Clinically affected people may have the appearance of enlarged bone, bowed extremities, shortened stature, and simian posturing because the body’s usual system for maintaining strong and healthy bone malfunctions. Normal bone turnover is altered in the affected areas. The resorption process accelerates, and the repair process responds by building a heavy, thickened, and enlarged bone. Although the new bone contains normal or increased amounts of calcium, the material of the bone is disorganized, and the bone is structurally weak. The result may be pain, deformity, fracture, and arthritis.

Distribution and Incidence

The disease appears to have its greatest prevalence in Europe and in regions inhabited by European emigrants such as Australia, New Zealand, and areas of South America. For no apparent reason, the disease is distinctly uncommon in African blacks, Orientals, and inhabitants of India and Scandinavia. Where studied, the incidence ranges between 3.5 and 4.5 percent of the population in high prevalence regions of the world, with a high of 8.3 percent in a part of Lancashire, England, to a low of 0.4 percent in Sweden.