Familial odontogenic keratocysts. Report of 3 cases and review of Japanese dental literature.
Familial odontogenic keratocysts are described in this report. The Case 1 patient, who has 3 sisters, developed odontogenic keratocysts. The 2 younger sisters (Cases 2 and 3) also had odontogenic keratocysts, although the elder sister did not have any odontogenic cysts. The father of the patients had a history of removal of a jaw cyst, and the mother was found later to have malignant ameloblastoma. Besides the odontogenic keratocysts, the Case 1 patient had basal cell nevus, prominent frontal process, and ocular hypertelorism; the Case 2 patient had prominent frontal process; the Case 3 patient had prominent frontal process, ocular hypertelorism, and squint. All 3 sisters are suspected of being patients with the basal cell nevus syndrome. The Japanese dental literature concerning the basal cell nevus syndrome is reviewed.
Kamiya
Y
Y
Narita
H
H
Yamamoto
T
T
Kameyama
Y
Y
Maeda
H
H
Nakane
S
S
D000293:Adolescent; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis / Q000235:genetics*; D002280:Basal Cell Carcinoma / Q000235:genetics*; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007633:Keratins; D009807:Odontogenic Cysts / Q000235:genetics* / Q000473:pathology
D007633:Keratins