Basal Cell Nevus (Subscribe)  

Basal cell nevus syndrome is an autosomal dominant condition with complete penetrance and variable expressivity. It is characterized by five major components, including multiple nevoid basal cell carcinomas, jaw cysts, congenital skeletal abnormalities, ectopic calcifications, and plantar or palmar pits. Other features include a host of benign tumors, ocular defects,...

Gorlin syndrome
* autosomal dominant
* multiple nevoid basal-cell carcinomas
* odontogenic keratocysts (OKCs)
* rib + vertebral anomalies
* short metacarpal(s)

Basal Cell Nevus Syndrome BCNS NEVOID BASAL CELL CARCINOMA SYNDROME; NBCCS MULTIPLE BASAL CELL NEVI, ODONTOGENIC KERATOCYSTS, AND SKELETAL ANOMALIES FIFTH PHACOMATOSIS GORLIN SYNDROME GORLIN-GOLTZ SYNDROME HYDROCEPHALUS, COSTOVERTEBRAL DYSPLASIA, AND SPRENGEL ANOMALY, INCLUDED

The syndrome is an autosomal dominant with incomplete penetrance. It is an ectodermal disorder and is characterised by: Basal cell naevi, over upper torso and shoulders with a risk of malignant change, particularly over the face. Odontogenic cysts. Bifid ribs and occasionally spina bifida calcification of the falx and sometimes in other soft tissues There...

Gorlin syndrome is an autosomal dominant cancer. Patients with this rare syndrome often have several organ anomalies, many of which are subtle. Study of patients with Gorlin syndrome yields useful information about its neural development and carcinogenesis. Clinicians should be familiar with Gorlin syndrome because patients tend to develop multiple...

Introduction Clinical Differentials Workup Treatment Follow-up Pictures Bibliography Author: Daniel Berg, MD, Program Director of Dermatologic Surgery, Associate Professor, Department of Internal Medicine, Division of Dermatology, University of Washington 2005

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