Woman affected by Fried syndromeSynonyms

  • Hair/tooth/nail syndrome
  • Nail dysplasia with hypodontia
  • Ectodermal dysplasia Type 8

Fried Syndrome is a Rare Genetic Condition Characterized by


Teeth

  • Misshapen
  • Large
  • Irregular
  • Missing (hypodontia)

Nails

  • Dystrophic
  • Thin, flat fingernail plates

Hair

  • Thin body hair
  • Fine scalp hair
  • Thin scalp hair
  • Sparse scalp hair (in some patients)
  • Hairs can be painlessly plucked with little force.
  • Sparse or absent eyebrows
  • Sparse or absent eyelashes

Diagnosing Fried Syndrome

A physician can diagnose Fried syndrome on the basis of physical features. Symptoms to look for:

  • Abnormal nails
  • Missing teeth
  • Abnormal hair

Causes of Fried Syndrome

The specific cause for Fried syndrome has not been definitively identified.  Genetic testing is not currently available.

Fried Inheritance

Fried syndrome is an autosomal recessive disorder.

To have an autosomal recessive disorder, you must inherit two mutated genes, one from each parent. Recessive disorders are usually passed on by two carrier parents. A carrier’s health is rarely affected, but they have one mutated gene and one normal gene for the condition. Two carriers have a 25 percent chance of having an unaffected child with two normal genes, a 50 percent chance of having an unaffected child, who also is a carrier, and a 25 percent chance of having an affected child with two copies of the abnormal genes.

Learn more about Fried syndrome at Online Mendelian Inheritance in Man.