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J Indian Soc Pedod Prev Dent. 2008 Jan;26 Suppl 1:S23-5.

Pyknodysostosis: oral findings and differential diagnosis.

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Department of Oral Diagnosis and Oral Pathology, School of Dentistry, Federal University of Rio De Janeiro, Rio de Janeiro, Brazil.


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.

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