Ehlors Danlos Syndrome in Dogs

Collagen is the main connective tissue of the body that gives the skin and joints their strength, elasticity, and durability. When a genetic defect causes an abnormal structure of the collagen fibers, the skin and joints are weakened. This causes looseness in the joints that can lead to lameness, and severely fragile skin that is susceptible to frequent injuries from even minor scratches. Other body tissues can also be affected by this collagen deficiency, including the eyes, heart, diaphragm, perineum, and blood vessels. Severely affected puppies have a risk of death, but in its mild or moderate forms, it is not fatal and can be managed by a controlled environment.

Ehlers-Danlos syndrome, also called cutaneous asthenia, refers to a group of medical conditions that occur because of defects in collagen structure and production. These defects create fragile, thin, and stretchy skin that hangs loosely in folds and tears easily with little trauma. Skin tears can enlarge and become infected, and lead to scars and subcutaneous hematomas. This rare condition primarily affects English Springer Spaniels, but can be seen in other breeds.

Symptoms are seen from birth, and can vary considerably in severity. They include:

• Fragile skin
• Loose or stretchy skin
• Hyperextensible skin
• Hanging folds of skin
• Looseness in joints
• Connective tissue dysfunctions
• 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 and poor wound healing
• Extensive scarring from wound healing
• Increased infections
• Lameness
• Pregnancy complications, uterine rupture or prolapse in females
• Eye abnormalities
• Dry and fine hair
• Weakened blood vessels

Ehlers-Danlos syndrome is caused solely by an inherited gene that affects collagen production. Only one copy of the autosomal dominant gene is needed for a dog to be affected. If an unaffected dog is bred with an affected one, the offspring has a 50% chance of being affected with the syndrome as well.

While this condition mostly affects English Springer spaniels, other breeds seen with this syndrome are:

• Beagles
• German Shepherds
• Daschunds
• Boxers
• Saint Bernards
• Manchester Terriers
• Welsh Corgis

A suspicion of Ehlers-Danlos syndrome is based on symptoms and a history of your dog, which may include lacerations, abscesses, and thin scars. To come to a positive diagnosis, your veterinarian will use a skin extensibility test to assess the hyper-elasticity of skin, using the skin extensibility index as a reference. A skin biopsy may be submitted to study the collagen structure and density.

There is no available cure for this condition. Treatment involves management of wounds and lifestyle modifications to accommodate your dog’s fragile, lifelong condition.

Surgery may be required for large and deep tears as they occur, often with anesthesia. Small lesions may be resolved with laser therapy. Severe ulcerations might require skin grafting. If hygromas occur, they may require surgery as they can progress to inflammations, infections, abscesses and granulomas. While often resolved with needle aspirations, chronic hygroma cases may need draining, flushing, and the placement of a drain tube. Complications are a risk for any surgery with this condition due to the fact the blood vessels are weakened, and can include secondary infections, poor wound healing, and difficulty administering intravenous fluids.

Vitamin C supplements have shown improvements in symptoms and may be prescribed.

Lifestyle and housing modifications will be needed to ensure the safety of your dog, and will be discussed with your veterinarian.

Extensive lifelong care is needed for management of this condition, which causes some affected dogs to be euthanized.

At home, keep an eye on large or deep wounds or tears, and promptly seek medical attention for stitches before they enlarge. Removal or padding of rough or sharp objects will be needed in your dog’s environment, especially in the place your dog sleeps or rests. Though it will affect your dog’s quality of life, restriction will be needed on vigorous exercise, such as playing, sports activities, or even running in places with obstructions, such as wooded areas.

Prevent this syndrome by not breeding affected dogs. There is no genetic test available for this condition, so use associated symptoms to determine whether or not a specific dog should be bred. Animals that carry a mutated gene are likely to show symptoms, though the severity is variable.