INBDE (Integrated National Board Dental Examination) Practice Exam

Which syndrome is characterized by multiple KCOTs, multiple BCCs, calcified falx cerebre, and is fatal?

1. Gorlin Syndrome

2. Epstein's Condition

3. Bohn's Disease

4. Malassez's Disorder

The correct answer is: Gorlin Syndrome

Gorlin Syndrome is a rare genetic condition that is characterized by the presence of multiple Keratocystic Odontogenic Tumors (KCOTs), which are benign cysts in the jaw, and multiple Basal Cell Carcinomas (BCCs), which are skin cancers. In addition, individuals with this syndrome may also have a calcified falx cerebri, which is a calcification in the membrane that surrounds the brain. This syndrome is fatal because the BCCs can spread to other parts of the body and cause complications. The other options (Epstein's Condition, Bohn's Disease, and Malassez's Disorder) do not exhibit the same symptoms as Gorlin Syndrome and therefore are incorrect. Epstein's Condition, also known as Epstein Syndrome, is a rare genetic condition that affects blood cells and can cause bleeding disorders. Bohn's Disease, also known as Bohn's Nodule, is a benign cyst that can occur in the jaw or gum tissue. Malassez's Disorder, also known as Malassez's Trichloacanthoma, is a rare skin disorder that presents as a wart-like growth.