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Ectodermal dysplasia

• Overview
• Symptoms
• Treatment
• Prevention

Definition:

Ectodermal dysplasia is a hereditary condition characterized by abnormal development of the skin, hair, nails, teeth, and sweat glands.

Alternative Names:

Anhidrotic ectodermal dysplasia

Causes, incidence, and risk factors:

There are many different types of ectodermal dysplasia. Multiple genes have been discovered that cause ectodermal dysplasias. The most common form of ectodermal dysplasia is linked to the X chromosome and usually affects men. Another form of the disease affects men and women equally.

Patients with ectodermal dysplasia may have absent or decreased sweating because of a lack of  sweat glands. Children with the disease may have difficulty controlling fevers. Mild illness may produce extremely high fevers, because their skin can't sweat and control temperature properly.

Affected adults are unable to tolerate a warm environment and require special measures to maintain a normal body temperature .

Patients may have chronic nasal infections with foul-smelling discharge and increased lung infections.

The skin is thin with light coloring. Hair may be absent or very thin. Teeth develop abnormally, and many teeth are missing.

• Review Date: 11/21/2005
• Reviewed By: Christos Ballas, M.D., Attending Psychiatrist, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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