Ectodermal Dysplasia, X-Linked (Dachshund Type)

Other Names: Hypohidrotic Ectodermal Dysplasia, XLHED
Affected Genes: EDA
Inheritance: X-Linked Recessive
Mutation: chrX:54509504 (canFam3): 1 bp deletion (del T)
Breed(s): Standard Smooth Dachshund

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Common Symptoms

X-linked ectodermal dysplasia is an inherited disease affecting dogs. Affected dogs are born with symmetrical hairlessness of variable severity on the head, lower back, lower chest, abdomen, and inner part of the limbs, closest to the body. Missing or abnormal sweat glands are typical and affected dogs are often missing teeth or display abnormal spacing between teeth. Affected dogs frequently display chronic nasal discharge and are more prone to respiratory and skin infections, which may be severe enough to be fatal or to warrant euthanasia.


Testing Tips

Genetic testing of the EDA gene will reliably determine whether a dog is a genetic Carrier of X-linked ectodermal dysplasia. X-linked ectodermal dysplasia is inherited in an X-linked manner in dogs meaning that female dogs must receive two copies of the mutated gene (one from each parent) to develop the disease while male dogs only require one copy of the mutated gene from the mother in order to develop disease. Therefore, male dogs more commonly present with symptoms of the disease. However, carrier female dogs may also present with mild symptoms. Each male pup that is born to a female dog known to be a carrier of X-linked ectodermal dysplasia has a 50% chance of inheriting the disease. Reliable genetic testing is important for determining breeding practices. In order to eliminate this Mutation from breeding lines and to avoid the potential of producing affected pups, breeding of known carriers is not recommended. Dogs that are not carriers of the mutation have no increased risk of having affected pups.


There may be other causes of this condition in dogs and a normal result does not exclude a different mutation in this gene or any other gene that may result in a similar genetic disease or trait.


References

  • Vasiliadis D, Hewicker-Trautwein M, Klotz D, Fehr M, Ruseva S, Arndt J, Metzger J, Distl O. A de novo EDA-variant in a litter of shorthaired standard Dachshunds with X-linked hypohidrotic ectodermal dysplasia. G3: Genes, Genomes, Genetics January 1, 2019 vol. 9 no. 1 95-104; https://doi.org/10.1534/g3.118.200814 [Not in PubMed]