Diagnostik und Therapie des Anti-p200-Pemphigoids

Pemphigoid diseases are a group of 7 different autoimmune diseases, characterized by autoantibodies directed to different proteins of the dermal-epidermal adhesion complex. These autoantibodies induce inflammation of the skin and mucous membranes, which can, consequently, elicit the emergence of blisters and erosions. The individual pemphigoid diseases exhibit a largely variable clinical presentation, but, at the same time, also closely resemble each other. Thus, thorough immunopathological analysis is often required to differentiate the diseases from each other. This is particularly the case for the differentiation between bullous pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. The latter is the most recently defined pemphigoid disease and the only pemphigoid disease whose autoantigen is still elusive. We present a typical case of anti-p200 pemphigoid and describe in detail the path of immunopathological diagnostics required to distinguish anti-p200 pemphigoid from other pemphigoid diseases.