title: Pyknodysostosis: an unusual presentation in a denture wearer. A case report.
abstract: Pyknodysostosis is a rare craniofacial syndrome characterized by cranial anomalies, dwarfism, osteopetrosis, hypoplasia of the angle of the mandible, abbreviated terminal phalanges, and dysodontiasis. The syndrome was first differentiated by Maroteaux and Lamy in 1962. Fewer than 130 cases have been described since that time. A 30-year-old Filipino man had most of the characteristics of the syndrome. Of particular interest is that he wore complete dentures and had a chief complaint of pain arising from partially erupting teeth under the denture. To our knowledge this is the first reported case from the Philippines.
authors.affiliation: Oral and Maxillofacial Surgery, College of Dentistry, University of the Philippines.
authors.forename: J V
authors.initials: JV
authors.lastname: Francisco
authors.forename: T J
authors.initials: TJ
authors.lastname: Nicholoff
mesh_terms: D000328:Adult; D003824:Denture, Complete; D006801:Humans; D008297:Male; D009087:Mucopolysaccharidosis VI / Q000601:surgery; D014078:Tooth Eruption; D014081:Tooth Extraction