Paget's disease of the Mandible

A 55-year-old man presented with a 2-year history of painful jaw enlargement and progressively ill-fitting dentures. He had no headaches or visual-field defects and did not have hyperhidrosis, oily skin, glucose intolerance, heart failure, or an increase in glove or shoe size.

The entire mandible was enlarged bilaterally to the angle of the jaw (Panels A and B), with marked misalignment of the upper and lower teeth. The serum level of insulin-like growth factor I was normal at 15.2 nmol per liter (normal range, 9 to 40), but levels of serum alkaline phosphatase and bone-specific alkaline phosphatase were elevated (154 IU per liter [normal level, <120] and 92 IU per liter [normal range, 15 to 41], respectively).

A bone scan revealed increased uptake of radionuclide in the jaw (Panel C); no other bones were involved. A mandibular biopsy confirmed the diagnosis of Paget's disease; there was no evidence of osteosarcoma.

Treatment with a bisphosphonate normalized the serum level of alkaline phosphatase. Earlier diagnosis and treatment might have limited further mandibular hypertrophy and pain, which the patient had for some time.