Background Pemphigus encompasses a group of life-threatening autoimmune bullous diseases
characterized by blisters and erosions of the mucous membranes and skin. Before the
era of immunosuppressive treatment, pemphigus was almost always fatal. Due to its
rarity, only few randomized controlled therapeutic trials are available. Recently,
rituximab has been approved as first-line treatment for moderate and severe pemphigus
vulgaris in Europe and the United States. Objectives TheAutoimmune blistering diseasesTask
Force of the European Academy of Dermatology and Venereology (EADV) has initiated
a throughout update of the guideline for the management of patients with pemphigus.
Results The guidelines for the management of pemphigus were updated, and the degree
of consent among all task force members was included. The final version of the guideline
was consented by the European Dermatology Forum (EDF) and several patient organizations.
