Goltz症候群(Focal Dermal Hypoplasia)の一例

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  • A Case Report for Goltz Syndrome

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We diagnosied a case of Goltz Syndrome in a 14 year old boy. Goltz Syndrome is a very rare disease which has the unique and characteristic clinical symptoms consisting of linear areas of dermal hypoplasia, abnormal skin pigmentation, and fawncolored nodules of adipose tissue in association with other congenital anomalies. There have been no more than 4 cases in Japan, and no report of oral involvement in this disease.<BR>The oral findings in this case were as follows:<BR>1) Hypoplasia of maxilla and mandible were observed.<BR>2) Marked hypoplasia of the enamel of nearly all permanent teeth, and tendency toward small tooth size were also observed.<BR>3) Absence or prolongation of permanent teeth eruption was observed.<BR>4) Verrucous papillomatous lesions were present around the lips and oi the palate.

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