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Curly Coat -- Rough Coat Syndrome in Cavalier King Charles Spaniels

A rarer but far more severe form of dry eye syndrome in some Cavalier puppies is a combination of dry eye and a congenital skin condition called "curly coat" or "rough coat" syndrome (ichthyosis keratoconjunctivitis sicca). The reference to a curly or rough coat comes from the unusually curly abnormality of the Cavalier's coat which is apparent at birth. However, the puppy also suffers from an extreme version of dry eye, and as the affected dog matures, it develops a deterioration of the skin which results in seborrhea, consisting of skin inflammation and excessive oiliness. Also, the dog's teeth, gums, and other connective tissues may be adversely affected. The form of dry eye associated with curly coat also is rare in that it is of congenital origin.

In cases of curly coat (rough coat) syndrome, nearly continuous daily care, including very frequent medicinal bathing, is required to treat the skin condition, as well as applying the eye medications. In a 2003 study reported by Dr. Keith C. Barnett, OBE MA PhD BSc DVOphthal FRCVS DIpECVO, European Specialist in Veterinary Ophthalmology, the dry eye condition of curly coat dogs may be so severe that cyclosporine therapy is ineffective, and the skin condition progresses into severe lesions. In a September 2006 paper, Dr. Barnett reported that successful treatment of the skin condition is not possible, although there can be some improvement in the dry eye condition.

Dr. Barnett reported that the need for constant care of the eyes and skin may lead breeders to resort to early euthanasia of the affected puppies as the only humane result, to avoid the dogs suffering from lifetimes of extreme discomfort and permanent eye damage.

No cases of the combination of dry eye syndrome and curly coat syndrome have been reported in any other breed. Studies have been conducted in the United States, United Kingdom, Australia, Sweden, the Czech Republic, and Iceland. It is believed to be due to a simple autosomal recessive gene. If so, then affected puppies are more likely to be found in cases of line breeding or inbreeding on carrier bloodlines. During a two year period recently in Iceland, more than half of many litters of Cavalier puppies were born affected by the syndrome. The disorder may be more widespread than previously believed as more owners and veterinarians become aware of its symptoms.

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Report of the Eye Sub-Committee - AGM 2006

ICHTHYOSIS KERATOCONJUNCTIVITIS SICCA (Dry eye/curly coat)

There have been 21 separate cases of the condition submitted to the AHT since the research started. This does not take into account the many undiagnosed cases that went unreported due to lack of knowledge. It is now believed that there were in the order of some 39 or more cases that the Michigan State University and the University of Tennessee were aware of. Several cases have occurred in Australia, Sweden and the Czech Republic. Iceland has one of the smallest gene pools and has been unlucky enough to have imported several carriers, to the point that in the period 2002-2003 there were instances of more than 50% of many litters being born affected. It seems to imply that the condition is more widespread that first though as more people come to recognise the symptoms.

Several theories have been suggested, but the most practical seems to be that the condition is inherited, probably by as simple autosomal recessive gene. This means that a percentage of the Cavalier population may be carriers and when two carriers come together and affected puppy could be seen. One would expect this to be in the ratio of one affected, two carriers and one clear. However, there have been numerous cases were half or nearly all of one litter have been affected. It is not yet known whether this is just co-incidence or some other form of inheritance at work. What has been established however is that in an affected puppy's pedigree, a suspected carrier is always identified on both the sire and dam's lines. It would seem that an affected puppy is more likely to be seen where line or inbreeding on carrier lines is carried out.

There is now sufficient pedigree data available and a genetic programme is being carried out by Dr Kathryn Mellersh, a well respected geneticist at the AHT. Twenty seven (27) candidate genes have been identified and the tests are currently in progress and final results are pending.

With written support from the CKCS Club an application was made the Kennel Club for a grant to help with the research into this condition. I am pleased to report that a grant of £4,200 has been approved by the Charitable Trust and payment has been made to the AHT.

This condition is not as widespread as some other health issues but is particularly nasty in its expression. There is no treatment for the condition, only symptom and pain relief management. It is considered by the researches to be a condition best dealt with by euthanasia as soon as it is recognised. I urge all breeders to make themselves familiar with the condition to prevent unnecessary suffering to afflicted puppies.

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