Index
- What is ectodermal dysplasia?
- Prognosis of the disease
- Symptoms of ectodermal dysplasia?
- Medical tests for ectodermal dysplasia
- What causes ectodermal dysplasia?
- Can it be prevented?
- Treatments for ectodermal dysplasia
- What specialist treats it?
What is ectodermal dysplasia?
Ectodermal dysplasias are disorders that cause abnormal development of the skin, teeth, hair, sweat glands or nails. In some cases patients with ectodermal dysplasia may also have a cleft palate or cleft lip.
This disease occurs when the outer layer of tissue, the ectoderm, of the embryo does not develop normally. That is, it has two or more structures derived from the ectoderm affected.
It produces an abnormal development of the skin, teeth,
hair, sweat glands, or nails
Prognosis of the disease
There is a normal life expectancy with early diagnosis and appropriate treatment.
However, if not diagnosed during infancy, it is possible that hyperthermia may cause severe brain damage.
Symptoms of ectodermal dysplasia
Some of the signs that may present include:
- Missing or abnormal teeth, or a smaller number of teeth.
- Cleft lip.
- Abnormal fingernails.
- Low nasal bridge.
- Reduced skin color.
- Thin and sparse hair.
- Large forehead.
- Hearing difficulties.
- weakened immune system
- Poor vision and decreased tear production.
In addition, patients have sweat gland deficiency, so they cannot sweat or sweat less than normal. For this reason, children have problems controlling fever and even a mild illness can cause a very high fever. Adults cannot tolerate a warm environment and need air conditioning and other measures to regulate a normal body temperature.
Medical tests for ectodermal dysplasia
To diagnose ectodermal dysplasia may be performed:
- X-rays of the teeth and bones.
- Biopsy of the mucous membranes.
- Genetic testing.
- Skin biopsy.
What are the causes of ectodermal dysplasia?
Each type of ectodermal dysplasia is caused by a mutation or change in a gene, that is, there has been an abnormal development of tissues or cells.
Sometimes it is hereditary, but in others it occurs without a family history.
Can it be prevented?
If there is a family history and you want to have children, genetic testing is recommended. It is also possible to diagnose ectodermal dysplasia while the baby is in the womb.
Treatments for ectodermal dysplasia
The disease has no specific treatment. It will be treated as symptoms appear. Treatment will depend on the age of the child.
For children, treatment may include full or partial dentures, and for older children, crowns or veneers are recommended. When primary teeth are replaced by permanent teeth, orthodontics may be needed to correct the position of the teeth and ensure their function.
In the case of adults, fixed restorations such as dental implants will be chosen, as well as oral surgery to improve dental arches.
Other measures include using artificial tears to prevent dry eyes, using a saline nasal spray to remove debris to prevent infection, and taking baths to regulate body temperature.
What specialist treats it?
Depending on the symptoms to be treated, a specialist in dermatology or dentistry will be consulted.