History and Geography

Paget's disease is not a new disease, having been suspected in a Neanderthal skull. Although isolated case reports in the mid-nineteenth century describe what is now called Paget's disease, the classical clinical description by Paget and a pathological description by Henry Butlin clarified this entity in 1876. Paget was a major figure in the medical community, having been knighted at the age of 43, at which time he began his observations of the first patient with the bone disease that would bear his name. He is also credited with having defined such diseases as Paget's disease of the breast, rectum, and skin; carpal tunnel syndrome; and trichinosis. Paget's disease of bone is unique in that it affects only adult humans; reports of Paget's disease in animals are not convincing. A childhood osseous condition called juvenile Pagefs disease appears to be a separate entity.

At present, it is difficult to reconcile the geographic isolation of this disease to Europeans (exclusive of Scandinavians) and their descendants. Theories of a slow virus infection in susceptible individuals seem dubious, for if this were the case, then theoretically the disease would have a more worldwide distribution. Isolated case reports of Paget's disease in South African blacks, Japanese, and Indians (from India) only serve to confuse the issue of transmission.

Temperature cannot be implicated, as high prevalence areas include disparate climatic regions of Italy, Spain, Germany, and eastern Russia. Similarly, occupation and degree of physical activity do not appear related to the cause of Paget's disease as the blend of geographic areas involves all types of lifestyles. Interestingly, the black population of the United States appears to sustain a higher frequency of Paget's disease than might be expected, given the rarity of the disease in Africa. An explanation in part involves intermarriage with those of European ancestry.

Roy D. Altman

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