Osteopoikilosis

OSTEOPOIKILOSIS (OSTEOPATHIA CONDENSANS DISSEMINATE; SPOTTED BONES)

Autosomal dominant inheritance, with loss of function mutation in LEMD3 gene.
Osteopoikilosis is the presence of multiple bone islands(enostosis)
Commonly found in association with osteopathia striata and Melorrheostosis, and some times considered to be a part of a spectrum of disease called, Mixed sclerosing Bone Dysplasia
White spots are seen in the skin in (disseminated lenticular dermatofibrosis)- Buschke-Ollendorf syndrome along with osteopoikilosis
Round, oval or lanceolate sclerotic lesions with their long axes parallel to the long axis of the bone. Often in the epiphysis and adjacent metaphysis
Lesions may involve any bone except the skull, ribs, and vertebrae
They usually line up along the trabeculae in a lanceolate fashion. In non trabecular areas they assume a more or less spherical shape.
Bony lesions of osteopoikilosis are stable over time and do not accumulate radionucleotide on bone scanning
There is no risk for malignant transformation, and hence no surgical intervention is required in typical presentations.

Osteopoikilosis is one of the “Do not touch” lesions as they are benign and no invasive investigation or procedure is necessary