ectodermal dysplasia: a case report and overview
Post on 14-Jul-2015
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THE CASE
sore throat, dryness of mouth(drinks frequently),
hyperthermia , heat intolerance in summer,
episodes of chest infection
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Ectodermal Dysplasia (ED)is a group of inherited developmt. abnormalities of
hair, nails, teeth, sweat glands and other ectodermal structures.
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Hypohydrotic ectodermal dysplasia is the most common.. approx.7000 cases worldwide - defective EDA,EDAR or EDARADD
signal receptors
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Detected delayed eruption of teeth self-reported symptoms from the hair, nails, sweat glands.
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Orofacial dysfunction .. chewing, swallowing,
xerostomia, speech
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Deficient malar region, saddle nose, longer chin,
retruded maxilla, collapse of face height
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
No sweating..intolerance to Sun.. Hyperthermia
- fatal in infants or brain damage
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Dry skin and eczema, nasal crusting, and
thick ear wax
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Other ectodermal derived glands-mammary gland,
thyroid, thymus, cornea, conjunctiva
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Lack of mucous glands - life threatening respiratory
infections
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Lack of subcutaneous fat -lean, low body weight,
low BMI
Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Nail dystrophy and ectrodactyly not seen in the
Hypohydrotic type
Denture problem discomfort d/t fragile oral mucosa repeated revisionslimited retention and stability
faster resorption of the hypoplastic bone
Implant problem inadequate alveolar bone
compromised osseointegration - hypoplastic bone
Xerostomia - dental caries
Early diagnosis - growth adapted measures extraction of primary teeth for favourable
eruption of existing permanent teeth
Orthodontic treatment as demanded by the prosthesis design
Tooth replacement composite-retained FDPs rather than conventional designs
Auto transplantation of permanent teeth into positions where teeth are missing
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