Hypohidrotic ectodermal dysplasia

Also known as

  • HED

Genetic disorder that affects the development of sweat glands, teeth, skin, hair and nails.

Condition details

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Photos of affected individuals

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Typical age range of first manifestation

  • Can manifest in Baby
  • Does not manifest in Toddler
  • Does not manifest in Child 4-10
  • Does not manifest in Child 10-18
  • Does not manifest in Adult

Symptoms

Baby
Lay terms Clinical terms
  • baby doesn’t sweat
  • baby feels hot all the time
  • baby with unexplained high temperatures
  • baby with breathing difficulties
  • baby keeps getting croup
  • baby with croup not getting better quickly
  • baby with bad smell coming from nose
  • baby with frequent nose bleeds
  • baby with no teeth
  • 1 year old with no teeth
  • baby with pointy teeth
  • baby with pointed teeth
  • baby with triangle shaped teeth
  • baby has a hoarse voice all the time
  • baby with blonde hair that sticks up
  • baby with eczema
  • baby with extremely dry skin
  • baby with dark shadows under eyes
  • Hypohidrosis
  • hyperthermia
  • absent teeth
  • malformed teeth
  • hypodontia
  • sparse scalp and body hair
  • hypotrichosis
  • peg-shaped teeth; prominent forehead
  • thick lips
  • flattened bridge of the nose
  • foul smelling discharge from nose (ozena)
Toddler
Lay terms Clinical terms
  • toddler still has no teeth
  • 2 year old with no teeth

Affected genes identified to date

  • EDA
  • EDAR
  • EDARADD

Inheritance patterns

Is X-linked Is Autosomal recessive

The majority of cases are X-linked (males only are affected / females are carriers).

There are cases of HED in which the inheritance is autosomal recessive (inherited from both parents)

Are carriers affected?

The majority of carriers are affected in one way or another. Carriers may sweat less than normal, have missing teeth or abnormal shaped teeth, have thinner hair than normal. Nipple formation may also be affected.

How many are affected?

Support groups and organisations

Ectodermal Dysplasia Society
Unit 1
Maida Vale Business Centre
Leckhampton
Cheltenham
Gloucestershire
GL53 7ER
Tel 01242 261332
Facebook

Known experts

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