Abstract
Basal cell nevus (Gorlin-Goltz) syndrome is a rare autosomal dominant disorder with multiple developmental anomalies and predisposition to various neoplasms. We present a 60 year old male with pigmented, ulcerated skin lesions in face, neck and trunk, histologically proved to be basal cell carcinomas. Mild exopthalmos, hypertelorism, chest wall deformity & scoliosis were noted. Radiological imaging showed calcification of falx cerebri & tentorium cerebelli, bridging of sella turcica, right third, fourth and fifth bifid ribs, scoliosis of lumbar spine, odontogenic keratocyst of mandible and flame shaped lucencies in hands. Nepalese Journal of Radiology / Vol.3 / No.1 / Issue 4 / Jan-June, 2013 / 94-98 DOI: http://dx.doi.org/10.3126/njr.v3i1.8821
Highlights
Case ReportA 60 years old male, presented with skin lesions in face, neck and trunk
There is history of sibling having similar skin lesions who died at the age of 15 years due to some unknown cause
On examination multiple pigmented ulcerated skin lesions were seen in the face neck & trunk
Summary
A 60 years old male, presented with skin lesions in face, neck and trunk. There is history of sibling having similar skin lesions who died at the age of 15 years due to some unknown cause. On examination multiple pigmented ulcerated skin lesions were seen in the face neck & trunk. Hypertelorism, chest wall deformity & scoliosis were noted. The pigmented lesions were shown to be Basal cell Carcinoma. Skull radiograph AP view shows calcification of falx (Fig. 1a). Axial NCCT scan shows heavily calcified falx cerebrii & tentorium cerebelii (Fig. 1b and 1c).Lateral view of skull shows bridging of sella turcica (Fig. 1d). Chest radiograph PA view shows bifid right fourth and fifth ribs & splaying of NJR / VOL 3 / No 1/ ISSUE 4 / Jan-June, 2013
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