Autoimmune bullous dermatoses

Autoimmune bullous dermatoses (BAID) are a heterogeneous group of about a dozen disorders characterized clinically by erosions and blisters and immunopathologically by autoantibodies against structural proteins of the skin or transglutaminase 2/3. The diagnosis of BAID has made enormous progress in the last decade due to the availability of standardized serological detection methods, which, knowing the clinical picture, allow diagnosis in the vast majority of patients. Through the development of various in-vitro and in-vivo-Models for the most common BAID, for example bullous pemphigoid, pemphigus vulgaris, mucosal pemphigoid and the rare epidermolysis bullosa acquisita, it is possible to identify key molecules and inflammatory pathways and to evaluate the effect of new anti-inflammatory therapies preclinically. The approval of rituximab for moderate and severe pemphigus vulgaris and the development of national and international guidelines for the most common BAID represents a significant advance in the care of these patients. Nevertheless, the limited therapeutic armamentarium is the greatest challenge for the management of BAID. However, several phase II and phase III randomized controlled clinical trials give reason to hope that that new effective and safe therapy options will be available in the next few years. This review article summarizes the epidemiology, clinic, diagnostics, pathophysiology and therapy of BAID and gives an outlook on the current diagnostic and therapeutic needs as well as future developments.