Disorder - Epidermolysis bullosa, dystrophic

Organ Systems Involved
Integument

Alternative disorders described within LIDA
    Epidermolysis bullosa junctionalis; epidermolysis bullosa simplex (epidermolytic epidermolysis bullosa)

Alternative Names
Dystrophic epidermolysis bullosa; congenital epidermolysis bullosa; epidermolysis bullosa congenital; mechanobullous skin disease; dermolytic epidemolysis bullosa

Presenting Signs
    This condition commonly appears as blistered skin. There have been cases reported of ulcerated foot pads, gums and tongue, and even the skin over the spine can be affected. Ulcers on the skin commonly occur around pressure points or where the skin is in close contact with bones. The nails can completely separate from the nail beds on the feet. The skin in general can be very fragile and separate from the layer below if it is knocked, and can be stretchy and easily torn.

    The condition is caused by a mutation in the genes that code for the formation of collagen fibres, a protein important in skin strength. This defect results in a reduction in the number and strength of the fibres. The condition develops soon after birth, and varies in severity and the area affected.

Cats at Risk
    Newborns, juvenile cats

Breeds Affected
Persian


Treatment
    No specific treatment recognised. Treatment of symptoms as they arise with the aim of improving quality of life is all that is available. An attempt can be made to reduce the possibility of trauma to affected cats and treatment of secondary infections can be undertaken where necessary. Problems are much more severe in this disease than in the similar disease, epidermolysis bullosa junctionalis, thus euthanasia is a common outcome.

PubMed References
Epidermolysis bullosa, dystrophic

Contributor
Tamara Butler