Journal of The Japanese Stomatological Society
Online ISSN : 2185-0461
Print ISSN : 0029-0297
ISSN-L : 0029-0297
Basal Cell Nevus Syndrome
Report of Four Cases in Two Families
Masato JIBIKIKoichi ASADAYoichi NAKAGAWATetsuo ASAYAMAYasuaki HASEGAWAKatsunori ISHIBASHI
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1994 Volume 43 Issue 3 Pages 452-455

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Abstract

Basal cell nevus syndrome is considered to be an autosomal dominant hereditary disease. But reports of this disease with comparison of symptoms between parent and child are rare. Four cases in two families with basal cell nevus syndrome and a review of 39 cases in 17 families with this disease were reported in Japanese literature. Four cases that we recently experienced were a case of a 47-year-old mother and her 14-year-old daughter and a case of a 47-year-old father and his 14-year-olddaughter. In these cases multiple jaw cysts, palmer and planter pits, calcification of the falx cerebri, bridging of sella turcica, frontal and biparietal bossing, ocular hyperterolism, broadened nasal root, and skeletal anomalies were observed. There were some symptoms similarities between parent and child.

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