Dermoodontodysplasia

background

Dermoodontodysplasia is one of several ectodermal dysplasias that affect the outer layer of a developing embryo. This layer, called the ectoderm, develops into the skin, hair, nails, teeth, and eyes. As suggested by its name, dermatoodontodysplasia is characterized by skin and dental problems.
Dermatoodontodysplasia is a rare condition. The exact incidence is unknown, and very few cases have been described in the scientific literature. It is believed to be an inherited condition, and was first described in a Brazilian family in which 11 individual cases occurred over four generations. Three more possible cases were reported in another family, and the affected siblings also had symptoms of other ectodermal dysplasias (hypodontia/nail dysgenesis and trichodermodysplasia).
Because ectodermal dysplasias involve abnormal development of the embryonic ectoderm, they may have similar symptoms. However, based on the symptoms in the known cases, dermatoodontodysplasia was determined to be distinct from other similar ectodermal dysplasias.
There is no cure for the condition. Instead, treatment focuses on the management of symptoms. There is limited information about the life expectancy and quality of life in individuals with dermoodontodysplasia.

Related Terms

Autosomal dominant disorders, ectodermal dysplasia, hypodontia, onychodysplasia, trichodermodysplasia, trichodysplasia.