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Mc cune albright syndrome - Clinicoradiological diagnosis of a rare case

Authors :
Anka Sharma
Anirudh Upmanyu
Vikrant O Kasat
Amit R Parate
Source :
Journal of Indian Academy of Oral Medicine and Radiology, Vol 33, Iss 1, Pp 95-98 (2021)
Publication Year :
2021
Publisher :
Wolters Kluwer Medknow Publications, 2021.

Abstract

Mc Cune Albright Syndrome (MAS) is a rare, sporadic disorder characterized by a triad of symptoms: fibrous dysplasia, cafe-au-lait spots, and endocrinopathy. It is thought to be caused by the mutation of the GNAS1 gene and is predominantly a disease of females. We hereby report a case of a 40-year-old man who presented with suppuration and mobility of teeth in the maxillary left posterior region. The patient also had a history of recurrent fractures of limbs since childhood. Clinical examination revealed asymmetry of the face, brownish-tan macules on the nape of the neck, back, and bilateral buccal mucosa as well as lower labial mucosa. The radiological investigation confirmed the presence of polyostotic fibro-osseous lesion while the biochemical investigations revealed endocrinopathy (hyperparathyroidism). This case report emphasizes the role of an oral physician in arriving at the diagnosis of a complex disorder like MAS.

Details

Language :
English
ISSN :
09721363
Volume :
33
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Journal of Indian Academy of Oral Medicine and Radiology
Publication Type :
Academic Journal
Accession number :
edsdoj.7a7f555bbd842c6aa0568bd2f084671
Document Type :
article
Full Text :
https://doi.org/10.4103/jiaomr.jiaomr_198_20