Gorlin - goltz syndrome: a case report
DOI:
https://doi.org/10.15218/zjms.2012.0027Keywords:
Basal cell carcinoma, Gorlin syndrome, Nevoid basal cell carcinoma, Odontogenic keratocystsAbstract
Gorlin-Goltz syndrome, also known as basal cell nevus syndrome, is an uncommon, autosomal dominant inherited disorder, which is characterized by numerous basal cell carcinoma, odontogenic keratocysts and musculoskeletal malformations. However, medical literature documents both common and lesser known manifestations involving the skin, central nervous system, craniofacial anomalies etc. A case of Gorlin –Goltz syndrome with no family history has been reported here, the patient have multiple odontogenic keratocysts, tiny pits involving the palms and soles, rib anomalies, numerous nevi and nodes predominantly present on his face, chest and back. Histopathological study confirms the presence of multiple keratocysts in the jaws and the absence of basal cell carcinoma of the skin. Immunohistochemical study showed negatively p53 staining result. Early diagnosis and treatment is important to prevent long term sequelae including malignancy and oromaxillofacial deformation and destruction.
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Copyright (c) 2012 Ahmad Haidar, Amera Kamal Khaleel, Layla Muhammad (Author)
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