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Nevoid basal cell carcinoma syndrome

Author(s): Kannan Karthiga | Sundharam Sivapatha | Manikandan R

Journal: Indian Journal of Dental Research
ISSN 0970-9290

Volume: 17;
Issue: 1;
Start page: 50;
Date: 2006;
Original page

Keywords: Odontogenic Keratocyst | Bifid ribs | Spina bifida | Basal cell Carcinoma | Kyphoscoliosis

Binkley and Johnson first reported this syndrome in 1951. But it was in 1960, Gorlin-Goltz established the association of basal cell epithelioma, jaw cyst and bifid ribs, a combination which is now frequently known as Gorlin-Goltz syndrome as well as Nevoid Basal Cell Carcinoma Syndrome (NBCCS). NBCCS is inherited as an autosomal dominant trait with high penetrance and variable expressivity. NBCCS is characterized by variety of cutaneous, dental, osseous, opthalmic, neurologic and sexual abnormalities. One such case of Gorlin-Goltz syndrome is reported here with good illustrations.
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