Curriculum · Musculoskeletal / Orthopedic / Rheumatology
Paget disease of bone
What it is
Paget disease of bone is uncontrolled bone turnover. It is a disorder of osteoclasts: excessive osteoclastic resorption is followed by increased osteoblastic activity. The skull, the spine and pelvis, and the long bones of the lower extremities are the sites most commonly affected. The name belongs also to diseases that are not this one: Paget's disease of the nipple, which is looked for on inspection in the breast examination, and extramammary Paget disease of the vulva.
Causes and risk
The predisposing factors are increasing age, male sex, and family history.
How it presents
The typical history is an older male with bone pain and an isolated raised ALP. The pain sits in the pelvis, lumbar spine and femur, and can begin as non-specific arthralgia and bony pains. A hat that fits tighter than before is a telling sign, since the skull is thickening. Classical untreated features are bowing of the tibia and bossing of the skull. Unilateral sensorineural hearing loss may be found on examination.