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Fibrous dysplasia of bone: craniofacial and dental implications
Author(s) -
Burke AB,
Collins MT,
Boyce AM
Publication year - 2017
Publication title -
oral diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.953
H-Index - 87
eISSN - 1601-0825
pISSN - 1354-523X
DOI - 10.1111/odi.12563
Subject(s) - fibrous dysplasia , gnas complex locus , craniofacial , mccune–albright syndrome , medicine , craniofacial abnormality , skull , bone disease , pathology , anatomy , osteoporosis , endocrinology , biology , genetics , psychiatry , precocious puberty , hormone , gene
Fibrous dysplasia ( FD ) is a rare bone disease caused by postzygotic somatic activating mutations in the GNAS gene, which lead to constitutive activation of adenylyl cyclase and elevated levels of cyclic AMP , which act on downstream signaling pathways and cause normal bone to be replaced with fibrous tissue and abnormal (woven) bone. The bone disease may occur in one bone (monostotic), multiple bones (polyostotic), or in combination with hyperfunctioning endocrinopathies and hyperpigmented skin lesions (in the setting of McCune–Albright Syndrome). FD is common in the craniofacial skeleton, causing significant dysmorphic features, bone pain, and dental anomalies. This review summarizes the pathophysiology, clinical findings, and treatment of FD , with an emphasis on the craniofacial and oral manifestations of the disease.