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Osteopoikilosis
Spotted Bone Disease

General Considerations

  • Rare autosomal dominant or sporadic osteosclerotic dysplasia
  • Multiple punctate sclerotic lesions representing "bone islands" or foci of compact bone located in cancellous bone
  • Asymptomatic
  • Occurs in the epiphyses and metaphyses with predilection for
    • Tubular bones of the hands and feet
    • Carpals
    • Tarsals
    • Pelvis
    • Scapula
  • Ribs, clavicles, spine, and skull are rarely involved
  • Usually clustered around joints
  • Males and females affected equally

Clinical Findings

  • Asymptomatic
  • Diagnosis is usually made incidentally

Imaging Findings

  • Well-defined sclerotic lesions clustered symmetrically around joints
  • The long axis of the lesion is typically lined-up with the long axis of the bone
  • Bone islands may have a thorny appearance
  • Low signal intensity on T1 and T2 weighted MRI images
  • Bone scan is normal

Differential Diagnosis

Treatment

  • None required

Complications

  • Associations may include connective tissue nevi called dermatofibrosis lenticularis disseminate which, along with osteopoikilosis, comprises the Buschke-Ollendorff syndrome
  • Also associated with keloid formation, dwarfism, spinal stenosis, dystocia, tuberous sclerosis and scleroderma
  • It may be related to osteopathia striata and melorheostosis

osteopoikilosis

osteopoikilosis

Osteopoikilosis. Black arrows point to numerous sclerotic bone islands surrounding the hip joints in a pattern characteristic of osteopoikilosis. CT images of the same patient show the well-circumscribed lesions in the femurs and pelvis.
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Osteopoikilosis: A Case Report. Khot R, Sikarwar JS, Gupta RP, Sharma GL. Ind J Radiol Imag 2005 15:4:453-454