Curly Coat Dry Eye Syndrome (CCDE): A Unique Inherited Condition in Cavaliers

Written By Megan Bowen

Among the inherited health conditions that affect Cavalier King Charles Spaniels, one of the most distinctive and serious is Curly Coat Dry Eye Syndrome (CCDE), also known by its medical name, Congenital Keratoconjunctivitis Sicca and Ichthyosiform Dermatosis (CKCSID).

Fortunately, this condition is relatively uncommon today thanks to the availability of DNA testing and responsible breeding practices. However, it remains important for Cavalier owners and breeders to understand what the condition is, how it affects affected dogs, and why genetic testing plays a vital role in protecting the future of the breed.

What Is Curly Coat Dry Eye Syndrome?

Curly Coat Dry Eye Syndrome is a severe inherited disorder found almost exclusively in the Cavalier King Charles Spaniel.

The condition is caused by a mutation in the FAM83H gene and is inherited in an autosomal recessive manner. This means that a puppy must inherit one copy of the defective gene from each parent to be affected.

Dogs that inherit only one copy are known as carriers. Carriers do not develop the disease themselves but can pass the mutation to their offspring.

Research has estimated that approximately one in eight Cavalier King Charles Spaniels carries the mutation responsible for the condition.

Why Is It Called Curly Coat Dry Eye Syndrome?

The name describes two of the most obvious features of the disease:

Curly Coat

Affected puppies are born with an unusual coat texture that differs significantly from the soft, silky coat expected in a healthy Cavalier.

The coat is often:

  • Curly or crinkled

  • Coarse to the touch

  • Dry and brittle

  • Sparse in some areas

  • Waxy or greasy in appearance

These coat abnormalities are usually noticeable from a very young age.

Dry Eye

Affected puppies are unable to produce normal tears. This results in a condition known as keratoconjunctivitis sicca, commonly referred to as "dry eye."

Unlike the more common form of dry eye seen in many breeds—which is often caused by immune-mediated disease, injury, medications, or environmental factors—dry eye associated with CCDE is congenital and present from birth.

What Causes the Condition?

The mutation affects the development and function of several body systems, including:

  • Tear glands

  • Skin

  • Hair follicles

  • Nails

  • Oral tissues

As a result, affected puppies often experience multiple health problems simultaneously.

What Are the Early Signs?

The earliest signs are usually visible within the first few weeks of life.

Coat Abnormalities

One of the first clues is the puppy's coat.

Owners or breeders may notice:

  • Curly or rough hair

  • Dry coat texture

  • Poor coat quality

  • Failure to develop the normal silky Cavalier coat

Poor Growth

Affected puppies are often:

  • Smaller than littermates

  • Slow to gain weight

  • Less robust than their siblings

Eye Problems

Once the eyes open, affected puppies may develop:

  • Thick eye discharge

  • Redness

  • Squinting

  • Excessive blinking

  • Signs of discomfort

Because they cannot produce adequate tears, the surface of the eye becomes dry and vulnerable to injury.

Other Clinical Signs

As affected dogs mature, additional symptoms often develop.

Skin Disease

Affected dogs commonly experience:

  • Dry, flaky skin

  • Thickened skin

  • Scaling

  • Crusting

  • Recurrent skin infections

The term ichthyosiform dermatosis refers to the fish-scale-like appearance that may develop in some affected dogs.

Nail Problems

Many affected dogs develop:

  • Brittle nails

  • Cracked nails

  • Abnormal nail growth

Dental Abnormalities

Dental problems are common and may include:

  • Missing teeth

  • Abnormally shaped teeth

  • Poor enamel quality

  • Early periodontal disease

Eye Ulcers

Because tears play a critical role in protecting and nourishing the eye, affected dogs are highly susceptible to:

  • Corneal ulcers

  • Chronic eye infections

  • Painful eye disease

  • Vision impairment

How Is CCDE Diagnosed?

Diagnosis is often based on a combination of:

  • Clinical signs

  • Physical examination

  • Ophthalmic examination

  • Skin assessment

  • DNA testing

The characteristic combination of coat abnormalities, dry eye, skin disease, and dental changes is often highly suggestive of the condition.

What Should You Do If You Suspect Your Cavalier May Be Affected?

If your puppy develops:

  • An unusually curly or coarse coat

  • Persistent eye discharge

  • Red or irritated eyes

  • Poor growth

  • Skin scaling or crusting

You should seek veterinary attention promptly. Referral to a veterinary ophthalmologist and, where appropriate, a veterinary dermatologist may be recommended.

Early diagnosis allows supportive treatment to begin before serious complications develop.

What Treatments Are Available?

Unfortunately, there is currently no cure for Curly Coat Dry Eye Syndrome.

Treatment focuses on managing symptoms and maintaining comfort.

Dry Eye Management

Affected dogs usually require lifelong eye treatment.

This may include:

  • Artificial tear replacements

  • Lubricating eye gels

  • Frequent eye medications

  • Treatment of secondary infections

Many dogs require eye drops several times each day throughout their lives.

Eye Ulcer Treatment

Corneal ulcers may require:

  • Antibiotic eye medications

  • Pain relief

  • Specialist ophthalmic care

  • Surgery in severe cases

Skin Care

Management may include:

  • Medicated shampoos

  • Moisturising products

  • Treatment of secondary infections

  • Regular veterinary monitoring

Dental Care

Affected dogs often require:

  • Frequent dental examinations

  • Professional dental treatment

  • Home dental care

What Is the Long-Term Outlook?

The prognosis varies depending on the severity of the condition and the success of ongoing management.

Many affected dogs require intensive lifelong care involving:

  • Frequent veterinary visits

  • Daily eye treatments

  • Ongoing skin management

  • Dental care

Despite dedicated management, quality of life can be significantly affected by the chronic nature of the disease.

For this reason, prevention through responsible breeding remains the most effective strategy.

The Importance of DNA Testing

One of the major successes in Cavalier health management has been the development of a DNA test for CCDE.

The causative mutation was identified in 2011, allowing breeders to accurately determine whether a dog is:

  • Clear (unaffected and not a carrier)

  • Carrier

  • Affected

DNA testing enables breeders to make informed mating decisions and avoid producing affected puppies.

Importantly, carriers do not need to be removed entirely from breeding programs. Instead, they can be bred responsibly to dogs that have tested clear, ensuring that no affected puppies are produced while maintaining valuable genetic diversity within the breed.

What About Crossbred Dogs?

Because the mutation originates in the Cavalier King Charles Spaniel, crossbred dogs that have Cavalier ancestry on both sides of their pedigree may also be at risk.

This highlights the importance of genetic testing regardless of whether the dogs involved are purebred or crossbred.

DNA Testing Providers

DNA testing for Curly Coat Dry Eye Syndrome is widely available through several laboratories, including:

  • Orivet (Australia)

  • Animal Health Trust (historically in the United Kingdom)

  • Other accredited canine genetic testing laboratories

Breeders should ensure testing is performed through reputable laboratories using validated testing methods.

Curly Coat Dry Eye Syndrome is a serious inherited disease unique to the Cavalier King Charles Spaniel. While affected dogs face lifelong challenges involving their eyes, skin, coat, nails, and teeth, the availability of a reliable DNA test has dramatically reduced the incidence of the condition in responsibly bred Cavaliers.

For owners, recognising the early signs and seeking prompt veterinary attention can help maximise comfort and quality of life for affected dogs. For breeders, DNA testing remains one of the most powerful tools available to prevent this devastating condition and safeguard the future health of the breed.

References

Forman, O. P., Penderis, J., Hartley, C., Hayward, L. J., Ricketts, S. L., Mellersh, C. S., & Vaudin, M. (2012). Parallel mapping and simultaneous sequencing reveals deletions in BCAN and FAM83H associated with discrete inherited disorders in a domestic dog breed. PLoS Genetics, 8(1), e1002462. https://doi.org/10.1371/journal.pgen.1002462

Lewis, T. W., Blott, S. C., & Woolliams, J. A. (2010). Genetic evaluation of inherited disorders in the Cavalier King Charles Spaniel. Veterinary Journal, 184(1), 27–32.

Mellersh, C. S. (2014). The genetics of eye disorders in dogs. Canine Genetics and Epidemiology, 1(3), 1–10. https://doi.org/10.1186/2052-6687-1-3

Merck & Co., Inc. (n.d.). Keratoconjunctivitis sicca in dogs. In Merck Veterinary Manual. https://www.merckvetmanual.com

Orivet Genetic Pet Care. (n.d.). Curly Coat Dry Eye Syndrome (CKCSID) in Cavalier King Charles Spaniels. https://www.orivet.com

Parker, H. G., Dreger, D. L., Rimbault, M., Davis, B. W., Mullen, A. B., Carpintero-Ramirez, G., & Ostrander, E. A. (2017). Genomic analyses reveal the influence of geographic origin, migration, and hybridisation on modern dog breed development. Cell Reports, 19(4), 697–708.

Slatter, D. H. (2018). Slatter's fundamentals of veterinary ophthalmology (6th ed.). Elsevier.

Veterinary Genetics Laboratory, University of California, Davis. (n.d.). Canine inherited disorders and DNA testing. https://vgl.ucdavis.edu

Megan Bowen
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