Pyknodysostosis: Oral findings and differential diagnosis

Abstract

Pyknodysostosis is a rare, genetic, autosomal recessive condition characterized by short stature, generalized bone sclerosis, and oral manifestations such as maxillary atresia and an increase of the mandibular angle. The main purpose of this article was to report a case of pyknodysostosis, describing the characteristic orofacial findings of the disease and discussing the differential diagnosis.

Keywords

• Dysostosis
• oral manifestations
• syndrome