Ectodermal Dysplasia: a case report and overview

HYPOHYDROTICECTODERMAL

DYSPLASIA

W. Robindro Singha case report by

THE CASE

A thin and diminutive 14 years old male of normal intellect in Icon Dental Clinic

THE CASE

Multiple missing teeth which he wanted to restore

THE CASE

sore throat, dryness of mouth(drinks frequently),

hyperthermia , heat intolerance in summer,

episodes of chest infection

THE CASE

Peg-shaped, conical teeth.

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

33 impacted(OPG)

THE CASE

Normally arched palatal vault

Underdeveloped alveolar bone

Ectopic hair

THE CASE

Fine, sparse hair in scalp

Non existent eyebrow

Ectopic hair

THE CASE

Fine brown eyelashes

No hair in other body parts

Little in armpit

Ectopic hair

THE CASE

Ectopic hair growth in malar region

THE CASE

Dry palm

Scaly skin around lips

THE CASE

Positive Iodine patch test

Nails are clinically not affected

THE CASE

Frontal bossing

Saddle nose

THE CASE

Everted lips

Pointed chin

Alloy coping and overdenture

Graft augmentation and implant supported

overdenture pending

THE CASE

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

Oligodontia determine the diagnosis

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

Altered cephalometry

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

Compassion and appropriate assimilation of clinical expertise and available treatment modalities greatly contribute to proper care