Gorlin-Goltz syndrome: Multiple cutaneous basal cell carcinomas, odontogenic keratocysts of jaw, palmar and plantar pits, rare medulloblastomas, and skeletal abnormalities
![image](https://i0.wp.com/basicmedicalkey.com/wp-content/uploads/2017/05/image02991.jpeg?w=960)
Scanning magnification view of a cutaneous keratocyst shows an undulating squamous epithelium lining the irregularly shaped cystic spaces. There are no associated sebaceous glands, unlike steatocystoma.
![image](https://i0.wp.com/basicmedicalkey.com/wp-content/uploads/2017/05/image02992.jpeg?w=960)
The cyst shows a bland, multilayered squamous lining surrounding an irregularly shaped cystic space.
![image](https://i0.wp.com/basicmedicalkey.com/wp-content/uploads/2017/05/image02993.jpeg?w=960)
High-magnification view of the lining of the cyst shows a bland, multilayered squamous epithelium with a dense eosinophilic cuticle
![image](https://i0.wp.com/basicmedicalkey.com/wp-content/uploads/2017/05/image02994.jpeg?w=960)
![image](https://i0.wp.com/basicmedicalkey.com/wp-content/uploads/2017/05/image02995.jpeg?w=960)
Another high magnification of a cutaneous keratocyst shows a bland squamous epithelium with a dense eosinophilic, jagged-appearing cuticle
![image](https://i0.wp.com/basicmedicalkey.com/wp-content/uploads/2017/05/image02996.jpeg?w=960)
ETIOLOGY/PATHOGENESIS
Genetic
• Most cases are associated with NBCCS (Gorlin-Goltz syndrome: Multiple cutaneous basal cell carcinomas, odontogenic keratocysts of jaw, palmar and plantar pits, rare medulloblastomas, and skeletal abnormalities)
Mutations for NBCCS in PTCH1 gene on chromosome 9q22.3-q31, which encodes receptor for the Sonic Hedgehog signaling pathway
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