Open AccessPycnodysostosis: Clinicoradiographic report of a rare case
Author(s) -
Mayur D. Dhameliya,
Ajit D Dinkar,
Manisha Khorate,
Sapna Sada Raut Dessai
Publication year - 2017
Publication title -
contemporary clinical dentistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.289
H-Index - 21
eISSN - 0976-237X
pISSN - 0976-2361
DOI - 10.4103/ccd.ccd_1105_16
Subject(s) - pathognomonic , osteosclerosis , medicine , phalanx , gonial angle , aplasia , short stature , dermatology , anatomy , pathology , radiology , radiography , disease , pediatrics
Pycnodysostosis is an uncommon autosomal recessive sclerosing bone disorder which is characterized by short stature and generalized diffuse osteosclerosis. Patients usually have a large head with separated sutures, open fontanels, aplasia of frontal sinuses, obtuse mandibular gonial angle, and acroosteolysis of the distal phalanges. This case report showed a 25-year-old female with features pathognomonic of pycnodysostosis. The emphasis is mainly on the early diagnosis as it has an important role in the general health of such patients and prevention of complications.