Paget's disease of bone

Background
  • Characterized by areas of bone with excessive osteolytic activity and subsequent increased ostoblastic activity, resulting in disorganized bone formation
  • Results in osteolytic lesions and weakened bone
  • Eventually, affected bones may be abnormally enlarged
  • Exact etiology unknown, likely genetic component
  • Prevalence increases with age, more common in people of European descent

Clinical Features
  • Initially asymptomatic or mild pain
  • Bone pain at affected sites, often worse at night
  • Pathologic fractures
  • If skull involved, may report increased hat size, headache, decreased hearing

Differential Diagnosis

Bone tumors and their mimics

Malignant

Benign
  • Giant cell tumor
  • Chrondroblastoma
  • Enchondroma
  • Langerhans cell histiocytosis of bone
  • Osteoblastoma
  • Osteochondroma
  • Osteoid osteoma

Other

Evaluation
Paget's disease of right innominate bone.
Skull CT showing “cotton wool spots” typical of later stages of Paget’s disease.
  • Imaging to evaluate for fractures
    • Initial lesion may be destructive, radiolucent (especially in skull)
    • Involved bones may be expanded, abnormally dense
    • May have multiple fissure fractures in long bones
  • Alk phos typically very high
  • Serum calcium may be high

Management
  • Treat fractures
  • Pain control
  • Bisphosphonates (e.g. Zoledronate) are treatment of choice for patients with severe symptoms or advanced disease

Disposition
  • Discharge uncomplicated patients

See Also



References
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