Ectodermal Dysplasia

Ectodermal Dysplasia

Ectodermal dysplasia is a group of congenital, non-progressive inherited disorders that has primary abnormality in structures of ectodermal origin such as skin, hair, nails, teeth, or sweat glands. It is a group of disease involving abnormalities in more than 2 of the above organs.

Causes and risk factors

Ectodermal dysplasia is caused due to genetic defect. Organs of ectoderm origin are bound to get affected.

Clinical presentation

Symptoms and signs of ectodermal dysplasia will differ according to the structure affected. Hair growth is slow and is fragile. The hair are curled or twisted. Hair may be thin, sparse, and light in color.  Both hand and toe nails can be thick and abnormal in shape. Nails are discolored and brittle. Cuticles are prone to infection. There is abnormal development of teeth. Congenitally missing teeth are seen. There is defective teeth enamel. Peg-shaped or pointed teeth are seen. Lack of perspiration resulting in poor temperature balance by body, frequent high temperature, and overheating in warm climates is observed.

Investigation

Medical history by the patient and clinical examination by the dermatologist helps in diagnosis. Biopsy of the mucous membranes and biopsy of the skin is required. Genetic testing is recommended.

Treatment

Treatment for ectodermal dysplasia is symptomatic and requires a team of doctors from various specialties. Supportive care is given such as use of wig and dentures to improve appearance. Use of artificial tears to replace normal tearing and prevent drying of the eyes. Spray the nostrils with saline nose spray often to remove debris and prevent infection. Staying in a cooler climate, taking cooling water baths, and use of air conditioning to keep a normal body temperature also helps.

Other Modes of treatment

The other modes of treatment can also be effective in treating ectodermal dysplasia.            Homoeopathy is a science which deals with individualization and considers a person in a holistic way. This science can be helpful in combating the symptoms. Similarly, the Ayurvedic system of medicine which uses herbal medicines and synthetic derivates is also found to be effective in treating ectodermal dysplasia.

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