Autoimmunity-driven enzymatic remodeling of the dermal-epidermal junction in bullous pemphigoid and dermatitis herpetiformis.

Pathogenesis of blister formation in bullous pemphigoid (BP) and dermatitis herpetiformis (DH) is associated with destruction of numerous components of the dermal--epidermal junction. Proteolytic enzymes (PE) are involved in a multitude of physiological reactions and may have impact on the epidermal--dermal integrity. Involvement of various PE in inflammation and blister formation in BP and DH is intensively investigated using both morphologic and functional approaches, particularly in BP. The development into the full-blown stage in BP and DH may be caused by an impairment of the human Fc receptor regulatory system that may cause the inefficiently controlled activation of inflammatory cells and subsequent secretion of various proteases.