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Basal Cell Nevus Syndrome

Known as: Gorlin Goltz Syndrome, Fifth Phacomatosis, gorlins syndrome 
An autosomal dominant genetic syndrome caused by abnormalities in the PTCH gene. It is characterized by multiple basal cell carcinomas at a young age… 
National Institutes of Health

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Aloi Tomasini Isaia syndrome
Autosomal dominant inheritanceBasal cell carcinomaBasal cell carcinoma, multipleBasal cell nevus

Papers overview

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Highly Cited
2004
Highly Cited
2004
δ‐Aminolevulinic Acid and Blue Light Photodynamic Therapy for Treatment of Multiple Basal Cell Carcinomas in Two Patients with Nevoid Basal Cell Carcinoma Syndrome
BACKGROUND Patients with nevoid basal cell carcinoma syndrome suffer from multiple basal cell carcinomas, requiring numerous… 
2003
2003
Eccrine porocarcinoma arising in a seborrheic keratosis evaluated with dermoscopy and treated with Mohs’ technique
A 78‐year‐old white woman returned for a routine 6‐month skin cancer examination. She had a history of actinic keratosis and… 
Highly Cited
2000
Highly Cited
2000
The Use of Imiquimod 5% Cream for the Treatment of Superficial Basal Cell Carcinomas in a Basal Cell Nevus Syndrome Patient
Background. Imiquimod 5% cream has been used effectively to treat superficial basal cell carcinomas (BCCs). Objective. The… 
1998
1998
No evidence for the H133Y mutation in SONIC HEDGEHOG in a collection of common tumour types
The human homologue of the Drosophila segment polarity gene patched is mutated in the cancer predisposition syndrome naevoid… 
Review
1996
Review
1996
Odontogenic keratocysts in the pediatric population.
OBJECTIVE To review the characteristics and treatment of odontogenic keratocysts in the pediatric population at our institution… 
1975
1975
The origin of keratocysts in the basal cell nevus syndrome.
Evidence is presented to support the validity of the basal cell hamartia hypothesis as an explanation for the histogenesis of jaw… 
Review
1969
Review
1969
Linear unilateral basal cell nevus with comedones.
A patient is presented and the literature of previously reported cases is reviewed, of a rare nevoid disorder which involves the… 
1968
1968
Basal cell nevus syndrome.
The manifestations of an inherited syndrome with emphasis on the roentgenographic findings have been described. Thirteen… 
Highly Cited
1965
Highly Cited
1965
PATHOLOGY OF THE NEVOID BASAL CELL CARCINOMA SYNDROME.
1964
1964
THE BASAL CELL NEVUS SYNDROME.
The basal cell nevus syndrome is a hereditary affection characterized primarily by multiple genetically determined basal cell… 
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