scholarly journals Gorlin's Syndrome: Case Report and Management Protocol

2009 ◽  
Vol 12 (1) ◽  
pp. 61-64
Author(s):  
R Rosti ◽  
A Irem ◽  
H Kayserili ◽  
S Yalcin

Gorlin's Syndrome: Case Report and Management ProtocolGorlin's syndrome (GS) is a rare autosomal, dominant syndrome, characterized by multiple basal cell carcinomas, odontogenic keratocysts, a characteristic facial appearance, skeletal anomalies and malignancies of various organs throughout the body. We describe a 14-year-old girl with GS and propose a management protocol.

2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Elizabeth A. Jones ◽  
Mohammed Imran Sajid ◽  
Andrew Shenton ◽  
D. Gareth Evans

Gorlin syndrome (Naevoid Basal Cell Carcinoma Syndrome) is a rare autosomal dominant syndrome caused by mutations in thePTCHgene with a birth incidence of approximately 1 in 19,000. Patients develop multiple basal cell carcinomas of the skin frequently in early life and also have a predisposition to additional malignancies such as medulloblastoma. Gorlin Syndrome patients also have developmental defects such as bifid ribs and other complications such as jaw keratocysts. We studied the incidence and frequency of basal cell carcinomas in 202 Gorlin syndrome patients from 62 families and compared this to their gender and mutation type. Our data suggests that the incidence of basal cell carcinomas is equal between males and females and the mutation type cannot be used to predict disease burden.


1998 ◽  
Vol 15 (2) ◽  
pp. 187-191 ◽  
Author(s):  
I. Lale Atahan ◽  
Ferah Yildiz ◽  
Enis Özyar ◽  
Dilek Uzal ◽  
Faruk Zorlu

Dermatology ◽  
2010 ◽  
Vol 221 (4) ◽  
pp. 300-302 ◽  
Author(s):  
B.A. Aigner ◽  
U. Darsow ◽  
M. Grosber ◽  
J. Ring ◽  
S.G. Plötz

2001 ◽  
Vol 27 (7) ◽  
pp. 667-669 ◽  
Author(s):  
Pelin Ekmekçi ◽  
Seher Bostanci ◽  
Rana Anadolu ◽  
Cengizhan Erdem ◽  
Erbak Gürgey

2005 ◽  
Vol 30 (5) ◽  
pp. 459-460
Author(s):  
R. NASSAB ◽  
S. RAYATT ◽  
F. PEART

This paper presents a rare hand presentation of Gorlin’s Syndrome, also known as Naevoid Basal Cell Carcinoma Syndrome, which has not been reported previously. This condition is an autosomal dominant, multisystem condition whose diagnosis is important to ensure further surveillance and treatment of features of the condition not routinely dealt with by hand surgeons. However, hand surgeons should be aware of this condition and those parts of the syndrome which may present to them.


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