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Ectodermal dysplasia
General information
• Represents a group of inherited diseases in
which two or more ectodermally derived
structure does not develop
• it can be autosomal dominant, autosomal
recessive and X-linked
• it shows X-linked inheritance
Clinical features
• It is characterised by hypotrichosis, hypohydrosis
and anhidrosis with saddle nose appearance
• the hair and scalp and eyebrows tend to be fine,
scanty and blond
• supraorbital and frontal bosses are pronounced
• there is heat intolerance. Such patients cannot
perspire and they usually suffer from
hyperpyrexia and inability to endure warm
temperature
Oral manifestations
• Oligodontia or anodontia
• The palatal arch is frequently high and the
cleft palate may be present
• Midfacial hypoplasia – there is protuberant lip
of the patient
Radiological features
• Since the alveolar process does not develop in
the absence of teeth, they appear as thin and
less vertical dimension
• Radiograph will show absence of teeth
Diagnosis
• Absence of teeth with dry skin is valuable aid
in the diagnosis of ectodermal dysplasia
• Reduction in the height of alveolar bone
Management
• Partial and complete denture should be
constructed for both functional and cosmetic
purpose
• Endogenous dental implant

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17.ectodermal dysplasia

  • 2. General information • Represents a group of inherited diseases in which two or more ectodermally derived structure does not develop • it can be autosomal dominant, autosomal recessive and X-linked • it shows X-linked inheritance
  • 3. Clinical features • It is characterised by hypotrichosis, hypohydrosis and anhidrosis with saddle nose appearance • the hair and scalp and eyebrows tend to be fine, scanty and blond • supraorbital and frontal bosses are pronounced • there is heat intolerance. Such patients cannot perspire and they usually suffer from hyperpyrexia and inability to endure warm temperature
  • 4. Oral manifestations • Oligodontia or anodontia • The palatal arch is frequently high and the cleft palate may be present • Midfacial hypoplasia – there is protuberant lip of the patient
  • 5. Radiological features • Since the alveolar process does not develop in the absence of teeth, they appear as thin and less vertical dimension • Radiograph will show absence of teeth
  • 6. Diagnosis • Absence of teeth with dry skin is valuable aid in the diagnosis of ectodermal dysplasia • Reduction in the height of alveolar bone
  • 7. Management • Partial and complete denture should be constructed for both functional and cosmetic purpose • Endogenous dental implant