Gorlin-Goltz syndrome: case report
DOI:
https://doi.org/10.23925/1984-4840.2018v20i1a14Keywords:
focal dermal hypoplasia, basal cell nevus syndrome, carcinoma, basal cellAbstract
Gorlin-Goltz syndrome (SGG) is a rare autosomal dominant disorder. Although it is hereditary, there are cases of spontaneous mutation. It is characterized by carcinogenic predisposition and several clinical manifestations. This article is about a 73-year-old white female patient with scoliosis, hypertelorism and four basal cell carcinomas (BCCs) on the back. The diagnosis of SGG was made. SGG is associated with clinical findings classified in larger criteria: minimum two BCCs in more than 20 years, or one in less than 20 years, keratocystic odontogenic tumor, palmoplantar pits, intracranial ectopic calcification, family history of SGG; and minor: craniofacial anomalies, macrocephaly, cleft or palatine lip, frontal bossa, hypertelorism, skeletal anomalies, ovarian fibroma, medulloblastoma. SGG is defined with the presence of two major criteria or one major and two minor. The treatment is multidisciplinary depending on the clinical manifestations of the patient and requires constant vigilance to new clinical findings.Downloads
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