Ectodermal Dysplasia, X-Linked (Shepherd Type)

Other Names: Anhidrotic Ectodermal Dysplasia, Congenital hypotrichosis, Ectodermal dysplasia, X-linked ectodermal dysplasia, X-linked hypohidrotic ectodermal dysplasia, XHED, XLED
Affected Genes: EDA
Inheritance: X-Linked Recessive
Mutation: chrX:54511433 (canFam3): G>A

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Common Symptoms

Ectodermal Dysplasia, X-Linked (Shepherd Type) is an inherited disease affecting dogs. This disease most commonly presents at birth. Affected dogs are born with symmetrical hairlessness on the forehead and the area over the lower back. Malformed and/or absent teeth (oligodontia) are also a feature of the disorder. Dogs may also have an inability to sweat (anhidrosis) due to missing or abnormal sweat glands. Tear production can be abnormal and dogs often develop dry eye (keratoconjuncitivitis sicca). Affected dogs are more prone to respiratory infections than other dogs and frequently have chronic nasal discharge. Prognosis for survival to adulthood is good. However, affected dogs should be monitored for signs of respiratory infections (cough, rapid or difficult breathing, and exercise intolerance) as they are sometimes fatal.

Breed-Specific Information for the Shiloh Shepherd

Shiloh shepherd is included as a breed susceptible to Ectodermal Dysplasia, X-Linked (Shepherd Type) because of its close relatedness to the German shepherd dog breed, which is known to develop this disease due to Mutation of the EDA gene. The frequency of the causal mutation in the general Shiloh shepherd population is unknown.

Testing Tips

Genetic testing of the EDA gene in Shiloh shepherds will reliably determine whether a dog is a genetic Carrier of Ectodermal Dysplasia, X-Linked (Shepherd Type). Ectodermal Dysplasia, X-Linked (Shepherd Type) is inherited in an X-Linked Recessive 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 the disease. Therefore, male dogs more commonly present with symptoms of the disease. Although much less common, carrier female dogs may present with mild symptoms. Each male pup that is born to a female dog known to be a carrier of Ectodermal Dysplasia, X-Linked (Shepherd Type) has a 50% chance of inheriting the disease. Female dogs that are not carriers of this Mutation have no increased risk of having affected pups. 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. Shiloh shepherds 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.


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