The nature of fibrous dysplasia
1 Department of Periodontology and Oral Medicine, School of Dentistry, Faculty of Health Sciences, University of Limpopo (Medunsa Campus), Pretoria, South Africa
2 Department of Oral Pathology, School of Dentistry, Faculty of Health Sciences, University of Limpopo (Medunsa Campus), Pretoria, South Africa
Head & Face Medicine 2009, 5:22 doi:10.1186/1746-160X-5-22Published: 9 November 2009
Fibrous dysplasia has been regarded as a developmental skeletal disorder characterized by replacement of normal bone with benign cellular fibrous connective tissue. It has now become evident that fibrous dysplasia is a genetic disease caused by somatic activating mutation of the Gsα subunit of G protein-coupled receptor resulting in upregulation of cAMP. This leads to defects in differentiation of osteoblasts with subsequent production of abnormal bone in an abundant fibrous stroma. In addition there is an increased production of IL-6 by mutated stromal fibrous dysplastic cells that induce osteoclastic bone resorption.