Congenital keratoconjunctivitis sicca and ichthyosiform dermatosis in 25 Cavalier King Charles spaniel dogs – part I: clinical signs, histopathology, and inheritance

Authors

  • Claudia Hartley,

    1. Unit of Comparative Ophthalmology, Centre for Small Animal Studies, Animal Health Trust, Lanwades Park, Kentford, Newmarket, Suffolk CB8 7UU, UK
    Search for more papers by this author
  • David Donaldson,

    1. Unit of Comparative Ophthalmology, Centre for Small Animal Studies, Animal Health Trust, Lanwades Park, Kentford, Newmarket, Suffolk CB8 7UU, UK
    Search for more papers by this author
  • Ken C. Smith,

    1. Department of Pathology and Infectious Diseases, Royal Veterinary College, Hawkshead Lane, North Mymms, Hatfield, Herts AL9 7TA, UK
    Search for more papers by this author
  • William Henley,

    1. Department of Statistics, School of Computing and Mathematics, University of Plymouth, Room 207, 2 Kirkby Place, Drake Circus, Plymouth, Devon PL4 8AA, UK
    Search for more papers by this author
  • Tom W. Lewis,

    1. Unit of Genetics, Centre for Preventive Medicine, Animal Health Trust, Lanwades Park, Kentford, Newmarket, Suffolk CB8 7UU, UK
    Search for more papers by this author
  • Sarah Blott,

    1. Unit of Genetics, Centre for Preventive Medicine, Animal Health Trust, Lanwades Park, Kentford, Newmarket, Suffolk CB8 7UU, UK
    Search for more papers by this author
  • Cathryn Mellersh,

    1. Unit of Genetics, Centre for Preventive Medicine, Animal Health Trust, Lanwades Park, Kentford, Newmarket, Suffolk CB8 7UU, UK
    Search for more papers by this author
  • Keith C. Barnett

    1. Unit of Comparative Ophthalmology, Centre for Small Animal Studies, Animal Health Trust, Lanwades Park, Kentford, Newmarket, Suffolk CB8 7UU, UK
    Search for more papers by this author

Address communications to:
C. Hartley
Tel.: +(0)1638 552700
Fax: +(0)1638 555600
e-mail: claudia.hartley@aht.org.uk

Abstract

The clinical presentation and progression (over 9 months to 13 years) of congenital keratoconjunctivitis sicca and ichthyosiform dermatosis (CKCSID) in the Cavalier King Charles spaniel dog are described for six new cases and six previously described cases. Cases presented with a congenitally abnormal (rough/curly) coat and signs of KCS from eyelid opening. Persistent scale along the dorsal spine and flanks with a harsh frizzy and alopecic coat was evident in the first few months of life. Ventral abdominal skin was hyperpigmented and hyperkeratinized in adulthood. Footpads were hyperkeratinized from young adulthood with nail growth abnormalities and intermittent sloughing. Long-term follow-up of cases (13/25) is described. Immunomodulatory/lacrimostimulant treatment had no statistically significant effect on Schirmer tear test results, although subjectively, this treatment reduced progression of the keratitis. Histopathological analysis of samples (skin/footpads/lacrimal glands/salivary glands) for three new cases was consistent with an ichthyosiform dermatosis, with no pathology of the salivary or lacrimal glands identified histologically. Pedigree analysis suggests the syndrome is inherited by an autosomal recessive mode.

Ancillary