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Case Reports
. 2013 Oct;4(4):547-50.
doi: 10.4103/0976-237X.123085.

Gorlin-Goltz syndrome: A rare case report

Chetan A Pol  1 , Suvarna K Ghige  2 , Ritesh R Kalaskar  2 , Suchitra R Gosavi  1
Affiliations
Case Reports

Gorlin-Goltz syndrome: A rare case report

Chetan A Pol et al. Contemp Clin Dent. 2013 Oct.

Abstract

Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder characterized by numerous basal cell carcinomas, odontogenic keratocysts (OKCs) and musculoskeletal malformations. A rare case of this syndrome observed in a 13-year-old male patient is presented in which multiple OKCs were causing disfigurement of the lower jaw as well as displacement and malocclusion of the teeth. Early diagnosis and treatment of this syndrome is important to reduce the severity of complications including cutaneous and cerebral malignancy and oromaxillofacial deformation and destruction due to jaw cysts.

Keywords: Calcification of falx cerebri; Gorlin-Goltz syndrome; Palmar pits; nevoid basal cell carcinoma syndrome; odentogenic keratocyst.

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Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
Intraoral photograph showing missing permanent teeth and deciduous teeth were still present
Figure 2
Figure 2
Orthopantogram showing three cystic lesions in the mandible and two involving the maxillary sinus on either side with the displaced permanent teeth
Figure 3
Figure 3
Axial computed tomography brain image showing bilamellar calcification of the falx cerebri
Figure 4
Figure 4
Side profile photograph showing mandibular prognathism (Class III jaw relationship)
Figure 5
Figure 5
Numerous palmar pits
Figure 6
Figure 6
Very thinned out labial cortical plate and displaced right permanent canine were seen
Figure 7
Figure 7
Histopathological photomicrograph showing uniform parakeratinized epithelial lining with satellite cyst in the connective tissue wall (H and E, ×10)
See this image and copyright information in PMC

References

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