What is Dermal Fragility Syndrome?
Skin and joints owe their elasticity and strength to collagen, the connective tissue found throughout the body. If those collagen fibers are abnormally structured due to a genetic defect, it can cause the skin and joints to lose that durability and become weakened. The result is very fragile skin that can be damaged from minor scratches and loose joints that are susceptible to lameness. Besides the skin and joints, other body tissue can be affected, including blood vessels, heart, diaphragm, perineum, and the eyes. While this condition is not usually fatal and can be managed through a controlled environment, puppies can be severely affected and have a higher risk of death.
Dermal fragility syndrome, also called cutaneous asthenia, dermatosparaxis, or Ehlers-Danlos syndrome, refers to a group of conditions that result from defects in collagen structure and production. The thin, stretchy, and fragile skin that affects dogs with these defects hangs loosely in folds and is prone to tear with very little trauma. These skin tears can become infected, leading to scarring and hematomas under the skin.
Symptoms of Dermal Fragility Syndrome in Dogs
Symptoms of dermal fragility syndrome are often seen from birth, and can vary from mild to severe conditions that involve the skin, joints, and sometimes other bodily tissues. The fragile condition of the skin can cause your dog to be more susceptible to skin injuries, which can then become enlarged or infected. Signs include:
- Fragile, soft, or thin skin
- Stretchy or loose skin
- Hyperextensible skin
- Hanging folds of skin
- Painful skin tears
- Skin bruising
- Subcutaneous hematoma, or hard blood masses under the skin
- Hygroma formations, or soft, fluid filled sacs that form over pressure points
- Delayed wound healing
- Extensive scarring from wound healing
- Increased infections
- Looseness in joints
- Connective tissue dysfunction
- Females are prone to pregnancy complications, uterine rupture or prolapse
- Eye abnormalities, such as cataracts and lens luxation
- Dry and fine hair
- Weakened blood vessels
Causes of Dermal Fragility Syndrome in Dogs
The cause of dermal fragility syndrome lies in an inherited gene that causes an abnormality in collagen structure and production, which then causes the flexibility and fragility seen in the skin and other tissues. This autosomal gene only needs one copy to be passed on to offspring to cause the condition to occur, meaning if an affected dog is bred with a healthy one, the puppies have a 50% chance of inheriting this syndrome. Dermal fragility syndrome seems to occur mostly in English Springer Spaniels, but have also been seen in other breeds, which include:
- Saint Bernards
- German Shepherds
- Welsh Corgis
- Manchester Terriers
Diagnosis of Dermal Fragility Syndrome in Dogs
Diagnosis of this syndrome is mainly based on the characteristic symptom of stretchy skin, and possibly skin wounds, abscesses, and thin white scars that result from tearing. After a physical exam, your veterinarian will assess the hyperelasticity of your dog’s skin using a skin extensibility test. A biopsy of the skin may be taken and analyzed for the structure and density of the collagen.
Treatment of Dermal Fragility Syndrome in Dogs
While there is no cure for dermal fragility syndrome, treatment aims to reduce injuries with lifestyle modifications, and manage any wounds that are incurred.
Keeping your home safe for your dog will prevent his skin from becoming torn and possibly infected, and can include such modifications as padding rough or sharp corners, your dog’s bedding, and any other obstacles that can injure him. Keeping him from playing with other animals, or running in wooded areas should also be avoided.
If your dog does sustain a skin injury, he will need to be treated. Laser therapy can be used on small tears, while large and deep ones may require surgery, or even skin grafting. Hygromas can lead to infections, abscesses and granulomas, and may also need surgery. Cases of chronic hygromas can be reduced through draining by way of needle aspirations, flushing, or a drain tube. Any surgery can carry risks for affected dogs, due to weakened blood vessels. Risks can include poor wound healing, secondary infections, and a difficulty in administering fluids intravenously.
There are no known medications that can help with this condition, although supplementing Vitamin C has shown improvement with symptoms.
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Recovery of Dermal Fragility Syndrome in Dogs
Dermal fragility syndrome is a lifelong condition that will need constant monitoring, care, and lifestyle changes. While not fatal, some dogs are euthanized due to the extensive care needed.
At home, be sure to remove or pad any sharp objects in your dog’s environment, especially in areas that he may rest or sleep. You may need to restrict activities such as sports, playing, or running in places with many obstructions. Monitor your dog for injuries, and treat any large wounds promptly before they get bigger or infected.
This syndrome can be prevented by careful breeding management. While no genetic test is available for the gene involved in this condition, dogs that have the gene often show symptoms, even if they are mild, and should not be bred.