Nevoid Basal-Cell Syndrome: literature review and case report in a family

• Main Authors: Alfio José Tincani, Antônio Santos Martins, Ricardo Gomes Andrade, Edgar José Franco Mello Jr., Marco Antônio Camargo Bueno
• Format: Article
• Language: English
• Published: Associação Paulista de Medicina
• Series: São Paulo Medical Journal
• Subjects: Basal-cell carcinoma; Skin tumors; Gorlin-Goltz Syndrome
• Online Access: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801995000300006&lng=en&tlng=en

The Nevoid Basal-Cell Carcinoma Syndrome (NBCC), or as it is also referred to, basal-cell nevus syndrome or Gorlin-Goltz syndrome, is characterized by multiple early-appearing basal cell carcinomas, keratocytosis of the mandible, and anomalies of the ocular, skeletal reproductive system. We describe four patients in the same family, all of them possessing a large number of skin tumors associated with other typical clinical and X-Ray anomalies of NBCC. The definitive treatment of NBCC has yet to be established, however, early diagnosis is very important as well as the periodical follow-up examination of ten patients, mainly due to the transformations in the skin lesions that may occur.