062 Granzyme B-mediated cleavage of hemidesmosomal proteins contributes to blister formation in epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita.

Autoimmunity to type-WI collagen is characterized by autoantibodies predominantly of lgG class to the non-collagenous domain of type-WI collagen present in the anchoring fibrils which bind basement membrane lamina densa to the anchoring plaques in the dermis. This results in a sublami.na densa split with a blister formation. Type WI collagen autoim.munity is heterogenous in its clinical spectru...

Epidermolysis bullosa acquisita: what's new?

Type VII collagen is an adhesion molecule of the extracellular matrix in epithelial basement membranes, and the main constituent of anchoring fibrils at the dermal-epidermal junction (DEJ). Autoimmunity against this protein is causing the rare organ-specific epidermolysis bullosa acquisita (EBA). EBA is a rare acquired, heterogeneous, chronic blistering disease of skin disease of skin and mucou...

The alternative pathway of complement activation is critical for blister induction in experimental epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita is a subepidermal blistering disease associated with tissue-bound and circulating autoantibodies against type VII collagen, a major constituent of the dermal-epidermal junction. The passive transfer of Abs against type VII collagen into mice induces a subepidermal blistering disease dependent upon activation of terminal complement components. To further dissect t...

Epidermolysis bullosa acquisita associated with psoriasis.

Epidermolysis bullosa acquisita and Crohn's disease.

A patient with epidermolysis bullosa acquisita (EBA) associated with Crohn's disease is presented. The clinical, histological and immunological findings were in keeping with previous reports. However, clinically normal skin and mucosa exhibited deposits of IgG and C3 in the basement-membrane zone. These deposits remained unchanged during the treatment period. It is therefore suggested that immu...