Current treatments and developments in pemphigoid diseases as paradigm diseases for autoantibody-driven, organ-specific autoimmune diseases

Pemphigoid diseases constitute a group of difficult-to-treat, remitting-relapsing autoimmune skin diseases. They are autoantibody-driven diseases with well-defined autoantigens in the hemidesmosomal complex at the dermal-epidermal junction (DEJ). Autoantibody deposition initiates the recruitment of granulocytes to the DEJ, where these cells degrade the hemidesmosomal complex, disrupting dermal-epidermal adherence, which clinically manifests as blisters and erosions. Due to their well-defined autoantigens and the location at the body surface, allowing the direct observation of inflammation throughout its course, pemphigoid diseases are excellent paradigm diseases to study the pathomechanisms of autoantibody-driven diseases.
Source: Seminars in Hematology - Category: Hematology Authors: Tags: April 2016 Suppl 1 ICIS Source Type: research