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Epidermolysis Bullosa Acquisita


Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal blistering disease of the skin and mucus membranes. Epidermolysis bullosa acquisita is caused by antibodies targeting type VII collagen, the major component of anchoring fibrils that connect the basement membrane to dermal structures. The classic presentation is characterized by blisters, mild mucosal involvement, and healing with dense scars primarily at trauma-prone areas. A second clinical presentation, the inflammatory form of epidermolysis bullosa acquisita, involves a generalized vesiculobullous eruption primarily on the trunk and flexural areas.

Epidermolysis bullosa acquisita is rare in humans. In animals, epidermolysis bullosa acquisita has been reported in dogs only. In canine epidermolysis bullosa acquisita, the immunoglobulin G (IgG) autoantibodies also target the type VII collagen noncollagenous (NC1) domain, which shares greater than 80% homology in amino acid sequence with the human NC1 domain.

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