Gorlin Goltz Syndrome – Case Series and Review of Literature.
Journal Title: IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) - Year 2019, Vol 18, Issue 4
Abstract
The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness It is characterized by disorders affecting multiple systems including skeletal, cutaneous, ophthalmic, reproductive, and nervous systems.. Gorlin syndrome is associated with germlinemutations in components of the Sonic Hedgehog pathway, including Patched1 (PTCH1) and Suppressor of fused (SUFU). The purpose of this present article is to describe the clinical, radiological, and histopathological findings in three cases of Gorlin Goltz syndrome diagnosed in our department, and to discuss the role of gene mutation analysis and circumstances to avoid in order to prevent primary manifestations.
Authors and Affiliations
Dr. Arun Devaraj, Dr. Sudha . S, Dr. Sujatha Varma, Dr. Navajeevraj MN, Dr. Suchithra KS
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