Jaffe-Lichtenstein syndrome - Report of a rare case
Journal Title: Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology - Year 2018, Vol 4, Issue 4
Abstract
Fibrous dysplasia FD is a benign slow growing skeletal disorder that causes swelling of the bone Lichtenstein described FD in 1938 as skeletal bone disorder which is distinguished by continuous replacement of normal bone with fibrous matrix FD is also associated with JaffeLichtenstein syndrome JLS and McCuneAlbright syndrome MAS JLS is characterized by polyostotic FD along with cafaulait pigmented lesions on the skin MAS presents with additional features of endocrine disorders along with the FD The disease is found to cause significant aesthetic problem and functional disturbance particularly those affecting the craniofacial skeleton Most often the clinical features and the classic radiographic presentation of the pathology would be sufficient to make a diagnosis However other fibroosseous lesions that mimic the radiographic features are critical to make a differential diagnosis for the clinician The present case report is about a 6 year old boy from south India who has been diagnosed as JaffeLichtenstein syndrome JLSKeywords Fibrous dysplasia McCuneAlbright Syndrome Cafeaulaithyperpigmentation LichtensteinJaffes disease Osteitisfibrosa alkaline phosphatase Acid phospatase Bone biopsy
Authors and Affiliations
Shaul Hameed. K, Laxmikant Chatra, Prasanth Shenoy, Anoop Kurian Mathew
Keywords
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- EP ID EP479811
- DOI 10.18231/2395-6194.2018.0047
- Views 136
- Downloads 0
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