Goltz-Gorlin syndrome: A rare genodermatosis
نویسندگان
چکیده
منابع مشابه
Gorlin-Goltz syndrome: A rare case report
Gorlin-Goltz syndrome (GS), also known as nevoid basal cell carcinoma syndrome, is an infrequent multisystem disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterised by keratocystic odontogenic tumours (KCOT) in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed ear...
متن کاملGorlin-goltz Syndrome: a Rare Case Report
Although odontogenic keratocysts are common in clinical practice, the simultaneous occurrence of multiple cysts in both the maxilla and mandible of a patient is rare. Gorlin-Goltz syndrome is an inherited autosomal dominant disorder with complete penetrance and extreme variable expressivity. It is characterized by keratocystic odontogenic tumors (KCOT) in the jaws, multiple basal cell nevi carc...
متن کاملGorlin-Goltz Syndrome
The Gorlin-Goltz syndrome (GGS) (the nevoid basal cell carcinoma syndrome-NBCCS) is a rare autosomal dominant syndrome caused due to mutations in the PTCH (patched) gene found on chromosome arm 9q. The syndrome, characterized by increased predisposition to develop basal cell carcinoma and associated multiorgan anomalies, has a high level of penetrance and variable expressiveness. GGS is a multi...
متن کاملGorlin-Goltz syndrome.
Gorlin-Goltz syndrome is a rare multisystemic disease inherited in an autosomal dominant pattern. It is characterized by numerous basal cell carcinoma of the skin, jaw cysts, and skeletal anomalies such as frontal bossing, vertebral anomalies, palmoplantar pits, and falx cerebri calcification. There is a tendency to tumors including medullablastoma, fibroma, rabdomyoma, leiomyosarcoma etc.. The...
متن کاملGorlin-Goltz Syndrome: Case Report of a Rare Hereditary Disorder
Introduction. Gorlin-Goltz syndrome is an inherited autosomal dominant disorder with complete penetrance and extreme variable expressivity. Case Report. The present paper highlights the importance of diagnostic criteria and histopathology in early and prompt diagnosis which will lead to proper treatment and genetic counseling of the patient. Discussion. Gorlin-Goltz syndrome is about multisyste...
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ژورنال
عنوان ژورنال: Indian Journal of Paediatric Dermatology
سال: 2014
ISSN: 2319-7250
DOI: 10.4103/2319-7250.143673