Ectodermal dysplasia
St. Luke's Hospital
Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
Find a Physician Payment Options Locations & Directions
Follow us on: facebook twitter Mobile Email Page Email Page Print Page Print Page Increase Font Size Decrease Font Size Font Size
America's 50 Best Hospitals
Meet the Doctor
Spirit of Women
Community Health Needs Assessment
Home > Health Information

Multimedia Encyclopedia

Ectodermal dysplasia

Definition

Ectodermal dysplasia is a group of conditions in which there is abnormal development of the skin, hair, nails, teeth, or sweat glands.

Alternative Names

Anhidrotic ectodermal dysplasia; Christ-Siemens-Touraine syndrome

Causes

There are many different types of ectodermal dysplasia.

Many gene defects can cause ectodermal dysplasias. The most common form of ectodermal dysplasia usually affects men. Other forms of the disease affect men and women equally.

Symptoms

People with ectodermal dysplasia may not sweat or may have decreased sweating because of a lack of sweat glands.

Children with the disease may have difficulty controlling fevers. Mild illness can produce extremely high fevers, because the skin cannot sweat and control temperature properly.

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

Other symptoms include:

  • Abnormal nails
  • Abnormal or missing teeth
  • Absent or decreased tears
  • Decreased skin color (pigment)
  • Heat intolerance
  • Inability to sweat
  • Large forehead
  • Lower than normal number of teeth
  • Low nasal bridge
  • Poor hearing
  • Poor temperature regulation
  • Poor vision
  • Thin, sparse hair
  • Thin skin

Exams and Tests

Tests that may be done include:

Treatment

There is no specific treatment for this disorder.

Some things you can do include:

  • Wear a wig and dentures to improve appearance.
  • Use artificial tears to replace normal tearing and prevent drying of the eyes.
  • Spray the nostrils with saline nose spray often to remove debris and prevent infection.
  • Live in a cooler climate and take cooling water baths or use water sprays to keep a normal body temperature (water evaporating from the skin replaces the cooling function of sweat evaporating from the skin).

Outlook (Prognosis)

Having ectodermal dysplasia will not shorten your lifespan, but you must pay constant attention to temperature regulation and other problems associated with this condition.

Possible Complications

  • Brain damage caused by increased body temperature
  • Seizures caused by high fever (febrile seizures)

When to Contact a Medical Professional

Call for an appointment with your health care provider if your child shows symptoms of this disorder.

Prevention

If you have a family history of ectodermal dysplasia and you are planning to have children, genetic counseling is recommended. In many cases it is possible to diagnose ectodermal dysplasia while the baby is still in the womb.

References

Morelli JG. Ectodermal dysplasias. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 648.


Review Date: 11/22/2011
Reviewed By: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com
 


Back  |  Top
About Us
Contact Us
History
Mission
Locations & Directions
Quality Reports
Annual Reports
Honors & Awards
Community Health Needs
Assessment

Newsroom
Services
Brain & Spine
Cancer
Heart
Maternity
Orthopedics
Pulmonary
Sleep Medicine
Urgent Care
Women's Services
All Services
Patients & Visitors
Locations & Directions
Find a Physician
Tour St. Luke's
Patient & Visitor Information
Contact Us
Payment Options
Financial Assistance
Send a Card
Mammogram Appointments
Health Tools
My Personal Health
mystlukes
Spirit of Women
Health Information & Tools
Clinical Trials
Health Risk Assessments
Employer Programs -
Passport to Wellness

Classes & Events
Classes & Events
Spirit of Women
Donate & Volunteer
Giving Opportunities
Volunteer
Physicians & Employees
For Physicians
Remote Access
Medical Residency Information
Pharmacy Residency Information
Physician CPOE Training
Careers
Careers
St. Luke's Hospital - 232 South Woods Mill Road - Chesterfield, MO 63017 Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
Copyright © St. Luke's Hospital Website Terms and Conditions  |  Privacy Policy  |  Patient Notice of Privacy Policies PDF Sitemap St. Luke's Mobile