How does Hypohidrotic ectodermal dysplasia happen?
Hypohidrotic ectodermal dysplasia has several different inheritance patterns. Most cases are inherited in an X-linked pattern and are caused by mutations in the EDA gene. A condition is considered X-linked if the mutated gene that causes the disorder is located on the X chromosome, one of the two sex chromosomes .
What body system does Hypohidrotic ectodermal dysplasia affect?
Hypohidrotic ectodermal dysplasia (HED) is a rare inherited multisystem disorder that belongs to the group of diseases known as ectodermal dysplasias. Ectodermal dysplasias typically affect the hair, teeth, nails, sweat glands, and/or skin.
What happens to a person with ectodermal dysplasia?
People with ectodermal dysplasia may not sweat or sweat less than normal because of a lack of sweat glands. In children with the disease, their bodies may have a problem controlling fevers. Even a mild illness can produce an extremely high fever, because the skin cannot sweat and control temperature properly.
What is Anhidrotic dysplasia?
Anhidrotic ectodermal dysplasia with immune deficiency (EDA-ID) is a form of ectodermal dysplasia, which is a group of conditions characterized by abnormal development of ectodermal tissues including the skin, hair, teeth, and sweat glands. In addition, immune system function is reduced in people with EDA-ID.
How does ectodermal dysplasia occur?
Ectodermal dysplasias are genetic disorders, which means that they can be passed on from affected people to their children. They are caused by mutations in various genes ; the mutations may be inherited from a parent, or normal genes may become mutated at the time of egg or sperm formation, or after fertilization.
When was Anhidrotic ectodermal dysplasia discovered?
The gene that causes x-linked hypohidrotic ectodermal dysplasia (XLHED), EDA, was discovered in 1996 by an international team of researchers.
What does Anhidrotic skin mean?
Anhidrosis is the inability to sweat normally. When you don’t sweat (perspire), your body can’t cool itself, which can lead to overheating and sometimes to heatstroke — a potentially fatal condition. Anhidrosis — sometimes called hypohidrosis — can be difficult to diagnose.
What are the causes of ectodermal dysplasia?
When does ectodermal dysplasia occur?
What is ectodermal dysplasia?
Ectodermal dysplasia is a hereditary disorder associated with abnormal development of embryonic ectodermally-derived organs including teeth, nails, hair and sweat glands. Hypodontia of the primary and permanent dentition is the most com-mon oral finding.
What does Anhidrotic mean?
Anhidrosis is the inability to sweat normally. When you don’t sweat (perspire), your body can’t cool itself, which can lead to overheating and sometimes to heatstroke — a potentially fatal condition.
What is the treatment for hypohidrotic ectodermal dysplasia?
Treatment of hypohidrotic ectodermal dysplasia may include special hair care formulas or wigs, measures to prevent overheating, removal of ear and nose concretions, and dental evaluations and treatment (e.g., restorations, dental implants, or dentures).