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Hereditary Cornification Disorders in Dogs

The skin's major structural barrier is provided by a few layers of keratinocytes that collectively comprise the epidermis. This barrier, known as the stratum corneum, is life-sustaining, prevents loss of water from the body and withstands chemical, microbial, immunological and ultraviolet/solar assault. The synthesis of the stratum corneum is often referred to as keratinization, but because this process involves more than keratins alone, cornification is the most complete term. Cornification represents the convergence of three biochemical processes, followed by desquamation: 1) the formation and organization of intracellular keratin intermediate filaments; 2) the formation and dispersion of an intercellular lipid glue or mortar that anneals the fully cornified cells together and makes the stratum corneum impermeable to many substances; and 3) the synthesis of the cornified envelope, the toughest portion of the stratum corneum that also functions to interconnect the intracellular keratin matrix with the intercellular lipid glue. Once keratinocytes have undergone complete cornification, they are called corneocytes.

Because keratins serve as the major cytoskeletal proteins of the epidermis, when the synthesis of a keratin protein is defective, the structural integrity of the affected keratinocytes and the stratum corneum they produce is affected.

Most disorders of cornification have a hereditary, metabolic or nutritional cause. The group of skin diseases with abnormal cornification includes:

Although well-characterized in man, abnormal cornification secondary to heritable superficial keratin defects is rarely reported in animals. Lesions are present at birth and pedigree analysis suggests an autosomal recessive mode of inheritance.

The disease has been described in Norfolk Terriers and Rottweilers that has all of the morphologic hallmarks of a keratinization disorder in humans. In these dogs, lesions are first noted shortly after birth when mild trauma (e.g. towel drying) results in superficial blister formation. As the dogs age, blisters are less conspicuous and hyperpigmentation and scaling, especially in frictional areas, are the major clinical findings.

Many features of this hereditary DOC correspond to the human condition CHILD syndrome. A major difference noted between superficial keratinization defects in humans and Norfolk terriers is that the canine disease appears to be recessive; whereas, as previously stated almost all keratinization disorders in humans are dominant.

Go Pets America recommends seeking the advice of your local veterinarian for the most appropriate vaccination program and for the diagnosis and treatment of your pet's health problems. For vaccination requirements please contact your state and local licensing authorities.