Cherubism

Synonyms

Familial fibrous dysplasia

Definition

Cherubism is a hereditary disease transmitted as an autosomal dominant trait with high penetrance and variable expressivity in different patients. The disease is characterized by bilateral expansion of the jaws caused by giant cell lesions.

Clinical Features

• Incidence

  The incidence of cherubism is unknown. However, 250 cases have so far been reported worldwide.

• Age

  Expansion of the jaws begins to be noticeable within the first few years of life, becomes progressively larger until puberty, and gradually resolves by middle age.

• Sex

  Higher penetrance has been reported in males.

• Site

  Initially, the lesions may present unilaterally, but they eventually involve both sides of the mandible, particularly at the molar and coronoid area. In the maxilla, the tuberosity is involved first, but anterior and orbital extensions may follow.

• Treatment

  Since the pathologic process in cherubism is self-limiting and can even be reversed by age, treatment is...