Abstract

PORTILLA, CC Ramírez et al. ABOUT A PATIENT WITH MULTIPLE LESIONS COMPATIBLE WITH GORLIN GOLTZ SYNDROME. Rev. argent. dermatol. [online]. 2018, vol.99, n.4, pp.78-86. ISSN 1851-300X.

Gorlin-Goltz syndrome (SGG) or nevoid basal cell carcinoma syndrome (SCBCN) is a monogenic genetic disorder with autosomal dominant inheritance, which has a mutation in the gene of the homologated protein zone 1 (PTCH1) located at position 21.32 of the long arm of chromosome 9 (9q21.32). It is characterized mainly by presenting: odontogenic cysts, multiple basal cell carcinoma or fissures at the level of the palms, the feet and skeletal alterations. We present the clinical case of a 6-year-old boy who attended the fourth month of life due to multiple small fissures in the palms and the feet. Histopathological study of the lesions was performed. The patient is attended in a comprehensive manner specialized in: Pediatrics, Dermatology, Nutrition, Maxillofacial Surgery, Dentistry and Genetics, taking frequent monitoring by external consultation so it would be the first case observed to date. In the same way, the results of histopathological exams were obtained for the confirmation of the diagnosis.

Keywords : syndrome; basal cell carcinoma; odontogenic cysts..