Epidermolysis bullosa in animals: a review.

[dystrophic epidermolysis bullosa]

Epidermolysis bullosa (EB ) is a hereditary mechanobullous disease of animals and humans , characterized by an extreme fragility of the skin and mucous membranes . The main feature of EB in humans and animals is the formation of blisters and erosions in response to minor mechanical trauma . Epidermolysis bullosa is caused by mutations in the genes that code for structural proteins of the cytoskeleton of the basal keratinocytes or of the basement membrane zone . Based on the ultrastructural levels of tissue separation , EB is divided into the following three broad categories : epidermolysis bullosa simplex , junctional epidermolysis bullosa and dystrophic epidermolysis bullosa . Human types of EB are divided into several subtypes based on their ultrastructural changes and the mode of inheritance ; subtypes are not fully established in animals . In humans , it is estimated that EB affects one in 17 ,000 live births ; the frequency of EB in different animals species is not known . In all animal species , except in buffalo with epidermolysis bullosa simplex , multifocal ulcers are observed on the gums , hard and soft palates , mucosa of the lips , cheek mucosa and dorsum of the tongue . Dystrophic or absent nails , a frequent sign seen in human patients with EB , corresponds to the deformities and sloughing of the hooves in ungulates and to dystrophy or atrophy of the claws in dogs and cats . This review covers aspects of the molecular biology , diagnosis , classification , clinical signs and pathology of EB reported in animals .

Diseases
Validation

Diseases presenting "mutations in the genes that code for structural proteins of the cytoskeleton of the basal keratinocytes or of the basement membrane zone" symptom

dystrophic epidermolysis bullosa

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