Ectodermal Dysplasia: a case report and overview

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HYPOHYDROTIC ECTODERMALDYSPLASIA W. Robindro Singha case report by THE ED SAGA

case report

Oligodontia THE CASE A thin and diminutive 14 years old male of normal intellect in Icon Dental Clinic case report

Oligodontia THE CASE Multiple missing teeth which he wanted to restore case report

Oligodontia THE CASEsore throat, dryness of mouth(drinks frequently), hyperthermia , heat intolerance in summer,

episodes of chest infection

case report

Oligodontia THE CASEPeg-shaped, conical teeth.

Only no. 13,16,22,23,26,34,43,44 were erupted

33 impacted(OPG) case report

Oligodontia THE CASENormally arched palatal vault

Underdeveloped alveolar bone case report

Ectopic hair

HypotrichosisTHE CASEFine, sparse hair in scalp

Non existent eyebrow case report

Ectopic hair

HypotrichosisTHE CASEFine brown eyelashes

No hair in other body parts

Little in armpit

case report

Ectopic hair

HypotrichosisTHE CASE Ectopic hair growth in malar region case report

HypohydrosisTHE CASEDry palm

Scaly skin around lips case report

HypohydrosisTHE CASEPositive Iodine patch test

Nails are clinically not affected case report

THE CASEFrontal bossing

Saddle nose case report

THE CASE Everted lips Pointed chin case report Alloy coping and overdenture

Graft augmentation and implant supported overdenture pending

THE CASE case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Ectodermal Dysplasia (ED)is a group of inherited developmt. abnormalities of hair, nails, teeth, sweat glands and other ectodermal structures. case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Hypohydrotic ectodermal dysplasia is the most common.. approx.7000 cases worldwide - defective EDA,EDAR or EDARADD signal receptors case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Detected delayed eruption of teeth self-reported symptoms from the hair, nails, sweat glands. case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Oligodontia determine the diagnosis case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Orofacial dysfunction .. chewing, swallowing, xerostomia, speech case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Deficient malar region, saddle nose, longer chin, retruded maxilla, collapse of face height case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Altered cephalometry case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

No sweating..intolerance to Sun.. Hyperthermia - fatal in infants or brain damage case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Dry skin and eczema, nasal crusting, and thick ear wax case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Other ectodermal derived glands- mammary gland, thyroid, thymus, cornea, conjunctiva case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Lack of mucous glands - life threatening respiratory infections case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Lack of subcutaneous fat - lean, low body weight, low BMI case report

Real-life Cullen: vampire teeth, pale skin..sensitive to sunFeatures can be devastating to mild !!THE ED SAGA

Nail dystrophy and ectrodactyly not seen in the Hypohydrotic type case report

Detecting the female carriersgenetic counselling of affected families optimize pediatric care of suspected male patientsprone to fever and respiratory infection case report

Detecting the female carriersPrenatal test diagnostic in suspected mothers Skin biopsy by fetoscopy (20 weeks Gestation) Linked markers on DNA from chorionic villi - safer case report

Detecting the female carriersEarly(prenatal) tests is unethical and controversial not an option by many familiesnormal intelligence and life expectancy usually case report Denture problem discomfort d/t fragile oral mucosa repeated revisions limited retention and stability faster resorption of the hypoplastic bone

dental challengesManaging missing teeth ..most important determinant of quality-life of the patient case report

Implant problem inadequate alveolar bone compromised osseointegration - hypoplastic bone Xerostomia - dental caries

dental challengesManaging missing teeth ..most important determinant of quality-life of the patient case report

Early diagnosis - growth adapted measures extraction of primary teeth for favourable eruption of existing permanent teeth

dental challengesManaging missing teeth ..most important determinant of quality-life of the patient case report

dental challengesOrthodontic 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 Managing missing teeth ..most important determinant of quality-life of the patient case report

dental challengesManaging missing teeth ..most important determinant of quality-life of the patient Compassion and appropriate assimilation of clinical expertise and available treatment modalities greatly contribute to proper care

Thank You

case report

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