Ectodermal Dysplasia

They consist of a complex group of diseases in which defects occur in at least two ectoderm derived structures: hair, teeth, nails and sweat glands. They may also be associated with anomalies of other organs. There are more than two hundred diseases described.

Many of them are hereditary with variable genetic transmission being autosomal dominant, autosomal recessive and X-linked inheritance.

Patients must be instructed to avoid hot environments and must be encouraged to use ventilation or refrigeration equipment.

Clothes must be light and fresh.When fever occurs, external cooling of the skin must be provided, as antipyretics do not work..

The use of emollients is always recommended, giving preference to less greasy options.

Dental care is important and dental prosthesis and implants may be recommended in adolescents and adults.

These children, as well as those affected by other genodermatoses, must be encouraged to play and attend school.

Information about Ectodermal Dysplasia

Definition and Transmission

They are a group formed by more than 200 diseases, all rare, in which changes occur in at least two of the following structures: hair, teeth, nails and glands producing sweat. Manifestations may appear soon after birth or in the first years of life. They are not contagious diseases and are usually transmitted from parents to children.


There are two main forms of ectodermal dysplasia: one in which the amount of sweat is decreased and another in which the amount of sweat is normal. Since childhood, patients have had heat intolerance, and may have fever which does not respond to antipyretic drugs. Hair can be fine and thin. Skin is usually dry and teeth may be absent or have altered shapes. Nails are usually fragile and thin.

Care and Treatment

They must be guided by physicians and specialized healthcare professionals. It is recommended to avoid hot environments, with low humidity, and to practice physical exercises in places which are not well ventilated or without refrigeration. It is recommended wearing light clothes and, if possible, installation of fans or air conditioning to keep the environment cool. It is also recommended the use of moisturizers once a day, after bathing.

Social Life

The children must be encouraged to live with other children and adults, attend school, play in well-ventilated environments, and they may even go swimming. As the disease is not contagious, there are no restrictions on attending public places.

The Reality of Ectodermal Dysplasia


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