Basal cell carcinomas, the most common human tumors, generally appear sporadically and in small numbers. Rarely, they may appear in great numbers and at an earlier age as a manifestation of the basal cell nevus syndrome, an autosomal dominant inherited disorder. Drawing on the retinoblastoma paradigm, we have begun a search for tumor suppressor genes important in the development of basal cell c...

Gorlin's syndrome, or basal cell nevus syndrome, is a relatively rare disease. It consists of a classic pentad of features comprised of multiple basal cell carcinomas, jaw cysts, calcification of the falx cerebri, pitting of the palmar and plantar surfaces and rib anomalies. A review of the clinical features, differential diagnosis, clinical work-up and current treatment is presented.

To cite: Nair PP, Khilari M, Peddawad D, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013200545 DESCRIPTION A 20-year-old woman presented with history of headache for 1 year. The headaches were mild to moderate in intensity, bilateral and non-throbbing, with no symptoms suggestive of raised intracranial pressure. The patient had hyperpigmented skin lesi...

An 11-year-old boy with multiple dentigerous cysts in the maxilla and mandible is described. Other findings seen in the face, plantar skin, skeletal system and oral cavity indicated the lesions to be due to the basal cell nevus syndrome. This was further confirmed by the presence of similar abnormalities in his father and brother.

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 associate...