Osteopoikilosis (OPK) is a rare, benign sclerosing bone dysplasia. It is often identified as an incidental finding on X-rays done for another reason.

Synonym: osteopathiacondensansdisseminata


Chromosome: 12q14.3

Gene: LEMD3 (LEM domain containing 3)


Inheritance: autosomal dominant, may be sporadic


Clinical features:

(1) often asymptomatic but may have bone and/or joint pain

(2) sclerosing bone dysplasia with disseminated, symmetrical hyperostotic areas, particularly in epiphyseal and metaphyseal regions of long bones

(3) variable findings: skin lesions, organ anomalies, endocrine dysfunction


Differential diagnosis:

(1) osteoblastic metastases to bone

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