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Osteopoikilosis and Its Clinical Significance: A Review of Literature

Rajat Rajkumar Jangir, Diwakar Laxmikant Mishra, Mohit Kumar, and Ashwini K Mathur

Osteopoikilosis is an asymptomatic osteosclerotic dysplasia initially described by Albers Schönberg in 1915. Osteopoikilosis is a rare disease with an estimated incidence of 1/50,000 and with an unknown etiology. Osteopoikilosis can have a very typical radiographic appearance and distribution; however, diverse clinical associated pathologies can lead to diagnostic uncertainty and the need for further investigation. We emphasize on the radiological characteristics of this condition with clinical presentations and associated dysplasias. The extensive search was conducted using an indexed search database (Pubmed and Cochrane) using MeSH terms, the relevant articles were chosen by title, abstract, language and publication date. The importance of its differential diagnosis is stressed with relevant review of the literature. When uniform multiple radio-dense round or oval sclerotic lesions in a periarticular distribution are found on radiographic examination, osteopoikilosis must be in the differential diagnosis. Osteopoikilosis should not be considered a coincidental radiographic finding, but rather part of a systemic disorder, as several developmental dysplasias coexisting with this disorder has been reported.Radiologically, the differential diagnosis are Enostosis, dysplasias of endochondral bone formation including osteopetrosis (Albers-Schönberg disease), pycnodysostosis and osteopathia striata (Voorhoeve disease), each with varying radiologic appearance. Although osteopoikilosis is generally considered an incidental finding, several developmental dysplasias coexisting with this disorder must be considered and excluded. In patients with a known or suspected malignancy, radionuclide bone scan has a critical role in distinguishing osteopoikilosis from osteoblastic bone metastases.