Abstract
Congenital generalized lipodystrophy (CGL) is a rare disease and is the result of an autosomal recessive gene made manifest by atrophy and disappearance of the systemic subcutaneous adipose tissue.
Growth promotion, hypertrichosis, cutaneous pigmentation, and insulin resistant diabetes mellitus are recognized as systemic findings in CGL. Although such pathogeneses are indistinct there are no fundamental therapies.
There have been no previous reports regarding the longitudinal changes in the disease.
In this study, the longitudinal changes and the oral findings of the CGL patients who attended our clinic, are reported. The oral findings observed were as follows.
1. Early calcification of the permanent teeth and early eruption 2. Enamel hypocalcification of the permanent teeth 3. Anomalies of the dentition and occlusion 4. Periodontal disease 5. Labial hypertrophy 6. Macroglossia and furrowed tongue 7. Uvula bifida 8. Palatal tonsil and adenoid hypertrophy
The present symptoms of gingivitis were noticeable, and the permanent dentition will soon be completed. An Orthopamtomogram revealed that the alveolar bone showed gradual absorption. The periodontal disease worsen with increased age, and mobility of the teeth was observed.
