A rare case of Hypohydrotic Ectodermal Dysplasia afflicting a 14 year old male is described.The characteristic features related to teeth and hair is also highlighted .

1. HYPOHYDROTIC
ECTODERMAL
DYSPLASIA
W. Robindro Singh
a case report by

2. THE CASE
A thin and diminutive 14 years old male of
normal intellect in Icon Dental Clinic

3. THE CASE
Multiple missing teeth which he wanted
to restore

4. THE CASE
sore throat, dryness of mouth(drinks frequently),
hyperthermia , heat intolerance in summer,
episodes of chest infection

5. THE CASE
Peg-shaped, conical teeth.
Only no. 13,16,22,23,26,34,43,44 were erupted
33 impacted(OPG)

6. THE CASE
Normally arched palatal vault
Underdeveloped alveolar bone

7. Ectopic hair
THE CASE
Fine, sparse hair in scalp
Non existent eyebrow

8. Ectopic hair
THE CASE
Fine brown eyelashes
No hair in other body parts
Little in armpit

9. Ectopic hair
THE CASE
Ectopic hair growth in malar
region

10. THE CASE
Dry palm
Scaly skin around lips

11. THE CASE
Positive Iodine patch test
Nails are clinically not affected

12. THE CASE
Frontal bossing
Saddle nose

13. THE CASE
Everted lips
Pointed chin

14. Alloy coping and overdenture
Graft augmentation and
implant supported
overdenture pending
THE CASE

15. 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.

16. 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

17. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Detected
delayed eruption of teeth
self-reported symptoms from
the hair, nails, sweat glands.

18. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Oligodontia determine
the diagnosis

19. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Orofacial dysfunction ..
chewing, swallowing,
xerostomia, speech

20. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Deficient malar region,
saddle nose, longer chin,
retruded maxilla,
collapse of face height

21. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Altered cephalometry

22. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
No sweating..intolerance to
Sun.. Hyperthermia
- fatal in infants or brain
damage

23. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Dry skin and eczema,
nasal crusting, and
thick ear wax

24. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Other ectodermal derived glands-
mammary gland,
thyroid, thymus,
cornea, conjunctiva

25. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Lack of mucous glands
- life threatening respiratory
infections

26. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Lack of subcutaneous fat -
lean, low body weight,
low BMI

27. Real-life Cullen: vampire teeth, pale skin..sensitive to sun
Nail dystrophy and ectrodactyly
not seen in the
Hypohydrotic type

31. Denture problem
discomfort d/t fragile oral mucosa
repeated revisions
limited retention and stability
faster resorption of the hypoplastic bone

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

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

34. 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

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