Hand-Schuller-Christian disease is characterized by proliferation of histiocytes with the classic triad including bone lesions, exophthalmus and diabetes insipidus. In the oro-facial region, clinical features such as gingivitis, jaw pain, jaw swelling, exfoliation and mobillity of teeth are reported.
We experienced a 13-year-1-month-old girl who had been sufferring from Hand-Schuller-Christian disease and healed at the age of 12 years. Her chief complaint was malocclusion with delayed eruption of parmanent teeth.
Her clinical findings were as follows:
1) Her hight and weight were bellow the -2 SD line in the cross sectional growth chart, and her bone age was delayed 2 years.
2) The profilogram revealed that the Middle face growth was retarded.
3) All the first permanent molars were unerupted.
4) The lower second premolars showed ectpic eruption.
5) Panoramic X-ray showed root dwarfism of_??__??__??_, malformation and hypoplasia of _??__??__??_. We suspected that these teeth anomalies had resulted from Hand -Schuller-Christian disease, because the period of the formation of these teeth was coincident with the time of the occurrence of her facial palsy and eczema.
6) Histopathological examination of the extracted lower right second molar and lower left second premolar showed partial enamel defects and irregular dentinal tubules.