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The ectodermal dysplasias are composed of a big and heterogeneous group of disorders that are inherited. These disorders are defined by primary defects in the development of no less than two tissues that come from the embryonic ectoderm. A lot of tissues can be included in this condition but the most often seen are the tissues of the skin, hair, nails, teeth and eccrine glands. According to the data, the first diagnosis of this condition that was reported happened in 1848. However, the actual term of the condition that is being used today was established in 1929.

What is ectodermal dysplasias

According to the experts, ectodermal dysplasias do not progress. However, they are congenital and diffuse. To this very day, the experts have described almost 200 distinct disorders that fall under the ectodermal dysplasias. Out of all the disorders, two are seen a lot more often that any of the other disorder. X-linked recessive hypohidrotic ectodermal dysplasia and hidrotic ectodermal dysplasia are the names of these two disorders. Nowadays, the doctors classify these dysplasias according to the clinical features. Certain defects only seen in the ectodermal structures are the main characteristics of pure ectodermal dysplasias. However, if the defects are accompanied by some other anomalies, a person is considered to be suffering from ectodermal dysplasia syndromes.

More than one cause of ectodermal dysplasias exists due to the fact that there are lots of different types of this condition. Gene defects are the cause in a lot of patients diagnosed with the condition.

Men are a lot more prone to being affected by the two most often seen forms of ectodermal dysplasias. However, other forms are seen in men as much as they are seen in women.

Decreased sweating or no sweating at all is two of the most often seen symptom of ectodermal dysplasias. The main reason why that is so, is because those affected by the condition do not have enough sweat glands. Fever is a symptom that occurs quite often as well because of the lack of sweat glands. When sweat cannot be made, the body has a problem to control the temperature the way it should. Apart from these two signs, there are lots of other signs like abnormal nails, missing teeth or teeth that are not considered to be normal, decreased tears, reduced skin color and thin skin, huge forehead, less teeth than it is considered to be normal, bad vision and hearing, thin hair and not tolerating the heat properly.

Complications of ectodermal dysplasias

Certain complications are possible in some cases of ectodermal dysplasias. Patients need to look out for them so that they do not become worse. A patient who suffers from serious dental abnormalities or cleft lip may experience certain feeding difficulties. That is troublesome because it may lead to malnutrition and even to the inability of expand. Those who have problems with anodontia can experience their bone that provides support to the denture to become smaller and smaller due to the use of denture for a prolonged period of time. Heat exhaustion and hyperpyrexia is a serious possibility for patients who are diagnosed with either anhidrosis or hypohidrosis. A person who is affected by chronic scalp dermatitis and erosions is more prone to battling bacterial and fungal skin infections that come back after being treated. It is not that uncommon for a person diagnosed with unnatural mucous gland function to become affected by some sort of a secondary infection. The upper respiratory tract is the most often infected area in these situations. Various viral, bacterial and fungal infections are more likely to be seen in those patients who are diagnosed with immunodeficiency.

Treatment options for ectodermal dysplasias

Before coming up with a treatment plan, the doctor needs to diagnose the condition properly. The diagnosis is usually done with the use of certain tests like a biopsy of the mucous membranes, biopsy of the skin and genetic testing.

The treatment plan is not the same as not the same ectodermal structures are affected in every patient.

Air conditioning helps a lot in cases of patients who suffer from either anidrosis or hypohidrosis. Consuming a lot of cool liquids is vital as well as it aids in maintaining of normal hydration and thermoregulation. Cool clothing is also an excellent option for these patients.

Those who are affected by dental defects need to get proper dental evaluations as early as possible as interventions as more possible at that time. Dental hygiene is of great significance in this situation. It is often seen that multiple replacements and dental implants are required. Surgery is an option for some patients. An early procedure can seriously better speech and reduce facial deformities in those affected by cleft lip or palate. Deformities of hand and foot can also be corrected with surgical procedures.

Talking to certain specialists like a dermatologist, general dentist, plastic surgeon, immunologist, genetic counselor and speech and physical therapists should be done as it will provide more options.

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