Pyknodysostosis: A Case Report

Pyknodysostosis is a rare bone disease that has autosomal dominant trait. it is character-ized by short stature, diffuse osteosclerosis, acro-osteolysis, finger, and nail abnormalities. other features include open fontanelles and sutures, relative proptosis and obtuse man-dibular gonial angle with relative mandibular prognathism. intraoral features such as den-tal abnormalities also have been reported. in this report, a case of pyknodysostosis in a 9-year-old boy is presented. most of the ob-served features were comparable to the other case reports but some findings such as re-peated chest infection, sleep apnea, kyphosis, scoliosis, root shortness, pulp narrowing, supernumerary and hypodontia could not be observed in this case. instead, in this patient features such as anterior open bite and decreased anterior tooth dis-play, not reported in other cases, was evident.