Rare Oral Diseases

                      

                           Rare Oral Diseases

Rare Oral abnormalities are dysfunctions and diseases of oral tissues and dentition caused by defective genes. Many genetic oral diseases indicate more complex disorders and are linked to inherited traits and defects, or result from spontaneous genetic mutations. Appropriate formation of the skeleton and dentition requires integration of various processes starting in early embryonic development. These include patterning of the head, limbs, and dental elements, cell movement and expansion, separation to specialized cells, matrix secretion, bio mineralization of bones and teeth, and remodelling of bone.

Types of Rare Oral Diseases

1. Hyperdontia:  This rare condition is affects approximately two percent of the population. In some cases, however, a person will grow extra teeth; this condition is known as hyperdontia. Those people affected with Hyperdontia typically have only one extra tooth that is usually located behind the upper, front teeth. This condition is thought to be hereditary but can be treated easily by removing the extra teeth.

2. Papillon-Lefevre Syndrome (PLS): PLS is a very severe deficiency in which an enzyme necessary to keep the connective tissues that support and connect the teeth. This is also a genetic disorder. Without this enzyme, a person with PLS will slowly lose his teeth. This starts with baby teeth by age four and adult teeth by early teens. Treatment with antibiotics has been known to slow the process.

3. Otodental Syndrome: This is a complex disorder with a strong genetic connection. In addition, it affects hearing and teeth. The canines and molars become enlarged, in such a way that the facial structure is affected. The other teeth are damaged. Treatment is frequently complicated and includes tooth extractions and repair.

4. Gingival Fibromatosis:  This causes an overgrowth of the gum tissue. The gums appear extremely swollen and puffy in a very abnormal way. It affects the teeth, may be in an oddly shaped or move around within the gums. The condition can be treated with oral surgery to recover the affected area. Some people have tissue re-growth, some don’t. So success for this disease is not constant.

Rare disorders are difficult to diagnose and are easily misdiagnosed; those of the skeleton are no exception. While progress has been made in our understanding of the underlying causes, pathologies, and mechanisms of bone diseases, those of the dental-oral-craniofacial (DOC) complex are frequently overlooked or superficially reported. This has implications in terms of understanding, diagnosing, and treating affected individuals, in turn affecting health and quality of life.