Ectodermal dysplasia

INTRODUCTION :

Ectodermal dysplasia syndrome is a large,heterogeneous group of inherited disorders , the manifestations of which could be seen in more than one ectodermal derivatives.

Common syndromes :
A. Hypohidrotic ED
B. Hidrotic ED

Etiology : 

 Ectodermal dysplasia syndrome results from aberant development of ectodermal derivatives in early embryonic life.

Symptoms

Teeth

In the development of tooth buds frequently result in congenitally absent teeth (in many cases a lack of a permanent set) and/or in the growth of teeth that are peg-shaped or pointed. The enamel may also be defective. Cosmetic dental treatment is almost always necessary and children may need dentures as early as two years of age. Multiple denture replacements are often needed as the child grows, and dental implants may be an option in adolescence, once the jaw is fully grown. Nowadays this option of extracting the teeth and substituting them with dental implants is quite common. In other cases, teeth can be crowned. Orthodontic treatment also may be necessary. Because dental treatment is complex, a multi-disciplinary approach is best.

People with ectodermal dysplasia may not sweat or may have decreased sweating because of a lack of sweat glands.

Children with the disease may have difficulty controlling fevers. Mild illness can produce extremely high fevers, because the skin cannot sweat and control temperature properly.
Affected adults are unable to tolerate a warm environment and need special measures to keep a normal body temperature.
Other symptoms include:

• Abnormal nails
• Abnormal or missing teeth
• Absent or decreased tears
• Decreased skin color (pigment)
• Heat intolerance
• Inability to sweat
• Large forehead
• Lower than normal number of teeth
• Low nasal bridge
• Poor hearing
• Poor temperature regulation
• Poor vision
• Thin, sparse hair
• Thin skin
  
  

  Treatment

  There is no specific treatment for this disorder.
  Some things you can do include:
  

  • Wear a wig and dentures to improve appearance.
  • Use 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.
  • Live in a cooler climate and take cooling water baths or use water sprays to keep a normal body temperature (water evaporating from the skin replaces the cooling function of sweat evaporating from the skin).
    
    Reference :
    Wikipedia , Shafers text book of oral pathology