EPIDERMOLYSIS BULLOSA ACQUISITA

Course overview

EPIDERMOLYSIS BULLOSA ACQUISITA (EBA) IS A RARE CHRONIC AUTOIMMUNE BLISTERING DISEASE OF THE SKIN AND MUCOUS MEMBRANES. EBA IS CAUSED BY AUTOANTIBODIES TO TYPE VII COLLAGEN, A MAJOR COMPONENT OF ANCHORING FIBRILS IN THE DERMAL-EPIDERMAL JUNCTION (DEJ). THESE ANCHORING FIBRILS ARE RESPONSIBLE FOR ATTACHING THE EPIDERMIS TO THE UNDERLYING DERMIS, AND BINDING OF AUTOANTIBODIES TO TYPE VII COLLAGEN SUBSEQUENTLY LEADS TO DETACHMENT OF THE EPIDERMIS, RESULTING IN SKIN FRAGILITY, BLISTERS, EROSIONS, SCARS, MILIA, AND NAIL LOSS. THIS ACTIVITY REVIEWS THE EPIDEMIOLOGY, PATHOGENESIS, AND CLINICAL FEATURES OF EPIDERMOLYSIS BULLOSA ACQUISITA AND DESCRIBES THE DIAGNOSTIC AND TREATMENT OPTIONS.