Nevoid basal cell carcinoma syndrome.
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.
Karthiga
Kannan S
KS
Department of Oral Medicine & Radiology, Sree Mookambika Institute of Dental Sciences, Padanilam, Kulasekharam. drskkomdr@yahoo.co.in
Sivapatha Sundharam
B
B
Manikandan
R
R
D000293:Adolescent; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008336:Mandibular Diseases / Q000175:diagnosis; D008439:Maxillary Diseases / Q000175:diagnosis; D008441:Maxillary Neoplasms / Q000175:diagnosis*; D009807:Odontogenic Cysts / Q000175:diagnosis; D012272:Ribs / Q000002:abnormalities; D014095:Tooth, Impacted / Q000175:diagnosis; D014097:Tooth, Unerupted / Q000175:diagnosis