Osteopoikilosis

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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 Osteoblastic metastatic disease  Osteopoikilosis is symmetrical, periarticular and the lesions are uniform in size Mastocytosis 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

Osteopoikilosis. Black arrows point to numerous sclerotic bone islands surrounding the hip joints in a pattern characteristic of ostepoikilosis. CT images of the same patient show the well-circumscribed lesions in the femurs and pelvis. For these same photos without the arrows, click here and here For more information, click on the link if you see this icon

Osteopoikilosis: A Case Report. Khot R, Sikarwar JS, Gupta RP, Sharma GL. Ind J Radiol Imag 2005 15:4:453-454