Anhidrotic ectodermal dysplasia; Christ-Siemens-Touraine syndrome; Anondontia; Incontinentia pigmenti
Ectodermal dysplasias is a group of conditions in which there is abnormal development of the skin, hair, nails, teeth, or sweat glands.
There are many different types of ectodermal dysplasias. Each type of dysplasia is caused by specific mutations in certain genes. Dysplasia means abnormal development of cells or tissues. The most common form of ectodermal dysplasia usually affects men. Other forms of the disease affect men and women equally.
People with ectodermal dysplasia have a lack of sweat glands. This means they may not sweat or sweat less than normal.
In children with the disease, their bodies may have a problem controlling fevers. When the skin cannot sweat, it is hard for the body to control temperature properly. So even a mild illness can cause a very high fever.
Adults with the disease can't tolerate a warm environment. Air conditioning and other measures are needed to keep a normal body temperature.
Depending on which genes are affected, other symptoms may include:
Tests that may be done include:
There is no specific treatment for this disorder. Instead, symptoms are treated as needed.
Things you can do may include:
These resources can provide more information on ectodermal dysplasias:
If you have a common variant of ectodermal dysplasia, this will not shorten your lifespan. However, you may need to pay attention to temperature changes and other problems associated with this condition.
If untreated, health problems from this condition may include:
Contact your health care provider if your child shows symptoms of this disorder.
If you have a family history of ectodermal dysplasia, and you are planning to have children, genetic counseling is recommended. Often, it is possible to diagnose ectodermal dysplasia while the baby is still in the womb.
Abidi NY, Martin KL. Ectodermal dysplasias. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 668.
Narendran V. The skin of the neonate. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 94.BACK TO TOP
Review Date: 7/1/2023
Reviewed By: Ramin Fathi, MD, FAAD, Director, Phoenix Surgical Dermatology Group, Phoenix, AZ. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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