17.ectodermal dysplasia
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Ectodermal dysplasia is a group of inherited diseases where two or more ectodermally derived structures are underdeveloped. It can be inherited in autosomal dominant, autosomal recessive, or X-linked patterns. Clinical features include sparse, fine hair and eyebrows, a saddle nose appearance, heat intolerance, and absence of sweat glands. Oral manifestations include missing teeth (oligodontia or anodontia), a high palate, and possible cleft palate. Radiographs show absence of teeth and a thin, less vertical alveolar process. Dentures and dental implants can be used to manage the condition for functional and cosmetic purposes.
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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