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Epidermolysis Bullosa
Epidermolysis bullosa syndromes are a group of hereditary congenital diseases in which blisters develop. It affects the attachments between the outer and inner layers of the skin. There are at least sixteen types of hereditary EB. The main human forms of epidermolysis bullosa (EB), namely EB simplex, junctional EB and dystrophic EB, have also been described in domestic animals (small and large ruminants, and horses) and companion animals (cats and dogs). Recently, dystrophic epidermolysis bullosa (DEB) has been described in Golden Retriever dogs. The several forms of this condition have been reported in Collie, Shetland Sheepdog, Toy Poodle, German Shorthaired Pointer, Golden Retriever, Akita and mixed breeds.
Signs
Blisters form and then rupture, leaving glistening, flat ulcers. They may be present at birth, or develop within the first week of life. The most severe blisters are on the feet, mouth, face, and genitals. In many cases the disease is fatal.
In DEB form, the disease is characterized by blisters and erosions in the mouth and esophageal lining, together with nails dystrophy and growth retardation. The skin lesions regress spontaneously in adult dogs, whereas the lining lesions persist, aggravate and spread, notably to the cornea.
Treatment
No curative treatment of human EB is currently available, and efforts are therefore being made to develop a gene therapy protocol in animals.
References
- The Merck/Merial Manual for Pet Health (Home edition)
- Bull Acad Natl Med. 2005 Jan;189(1):107-19; Characterization of a canine model of dystrophic bullous epidermolysis (DBE). Development of a gene therapy protocol. Magnol JP, Pin D, Palazzi X, Lacour JP, Gache Y, Meneguzzi G.
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