Ectodermal Dysplasia: Treatment, Cost and Side Effects

Ectodermal dysplasia is a group of syndromes that are hereditary and the symptoms are derived from abnormalities of the ectodermal structures. The syndromes are all non-progressive and affect the skin and a few other organs. The most fatal characteristic of this disorder is the suppressed immune system and defective immune glands due to which the respiratory tract is affected. There are more than 150 disorders in the group affecting the ectoderm which is the most exterior germ layer of the embryo. Symptoms of the disorders include abnormal hair growth because of defective hair follicles. The hair growth on the scalp and the other body parts are quite sparse, thin, light coloured, can be curly and very fragile. The pigment of the skin is also affected and thus the skin appears very light pigmented. Some patients have brown or red skin pigmentation and the skin can easily catch infection. There may be cracking, bleeding, rashes on the skin. The skin on the palms and soles may be very thick. The finger and toenails are hypo-pigmented, shaped abnormally, very thick and brittle and are characterised by very slow growth. One of the peculiarities of the disorders is that a person with ectodermal dysplasia cannot sweat because of abnormality of the sweat glands. This result in inability to regulate the temperature of one’s body, thereby, feeling overheated especially in the presence of high temperature. Hence, it is important that they remain in cool environment. Symptoms also include abnormal or absent teeth, or the teeth may be pointed or peg shaped with defective enamel. Children would require cosmetic dental treatment and may also require dental implants and multiple denture replacements. Abnormalities in other features like broader noses, pronounced chins and dryness in eyes thereby requiring usage of artificial tears are also common in people with ectodermal dysplasia. There is no cure for the disorders. Treatments are subjective of reducing the symptoms only. Patients may be prescribed with cosmetic corrections of facial or dental abnormalities, vaccines to protect against getting infectious disease, surgery for some defects of the body and so on.

Ectodermal dysplasia is a group of syndromes that are hereditary and the symptoms are derived from abnormalities of the ectodermal structures. The syndromes are all non-progressive and affect the skin and a few other organs. The most fatal characteristic of this disorder is the suppressed immune system and defective immune glands due to which the respiratory tract is affected. There are more than 150 disorders in the group affecting the ectoderm which is the most exterior germ layer of the embryo. Symptoms of the disorders include abnormal hair growth because of defective hair follicles. The hair growth on the scalp and the other body parts are quite sparse, thin, light coloured, can be curly and very fragile. The pigment of the skin is also affected and thus the skin appears very light pigmented. Some patients have brown or red skin pigmentation and the skin can easily catch infection. There may be cracking, bleeding, rashes on the skin. The skin on the palms and soles may be very thick. The finger and toenails are hypo-pigmented, shaped abnormally, very thick and brittle and are characterised by very slow growth. One of the peculiarities of the disorders is that a person with ectodermal dysplasia cannot sweat because of abnormality of the sweat glands. This result in inability to regulate the temperature of one’s body, thereby, feeling overheated especially in the presence of high temperature. Hence, it is important that they remain in cool environment. Symptoms also include abnormal or absent teeth, or the teeth may be pointed or peg shaped with defective enamel. Children would require cosmetic dental treatment and may also require dental implants and multiple denture replacements. Abnormalities in other features like broader noses, pronounced chins and dryness in eyes thereby requiring usage of artificial tears are also common in people with ectodermal dysplasia. There is no cure for the disorders. Treatments are subjective of reducing the symptoms only. Patients may be prescribed with cosmetic corrections of facial or dental abnormalities, vaccines to protect against getting infectious disease, surgery for some defects of the body and so on.

People with symptoms of ectodermal dysplasia like sparse, think hair, cleft lips or palette, wedge shaped teeth, abnormally shaped nails, lightly pigmented skin, unable to perspire etc are eligible for the treatment.

People not showing the symptoms of ectodermal dysplasia will not be eligible for treatment of ectodermal dysplasia. Certain deformities like cleft lip, or abnormally shape of nail or thin and sparse hair can be due to some other reasons also and can be acquired later in life also. Hence, such people will not be eligible for treatment for ectodermal dysplasia.

No there are no side effects of the treatments. The treatment options are all safe.

There are no such guidelines after treatment of ectodermal dysplasia because the treatments mainly deal with managing the symptoms rather than completely eradicating the disorders. So, the measures should be taken whenever the symptoms are visible and cause discomfort.

Ectodermal dysplasia is a group of disorders and cannot be cured completely. Only the symptoms can be managed and controlled using medications, surgery and some simple measures like keep the body cool and hydrated, checking skin and scalp infections, etc. Hence, managing and controlling the symptoms is an on-going process that will continue throughout life.

Treatment for ectodermal dysplasia costs between Rs. 200 to Rs. 2,000 based on the type of treatment irrespective of male and female of any age.

No, the results cannot be said to be permanent because only the symptoms are managed. There is no cure for the disease. So, a particular symptom can be eased once in life, but cannot be said that it will not recur. So, the symptoms on recurrence will require the treatments again.

No alternative research has been reported yet for ectodermal dysplasia.