Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV)

Abstract

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 The Autoimmune blistering diseases Task 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.

Rituximab in practice: Clinical evaluation of patients with pemphigus after rituximab administration

“…In case of any sign of relapse in the 4 to 6 months of RTX infusion, readministration of 2 g RTX may be suggested. However, in cases with disease recurrence during prednisolone tapering, before the fourth month, a dose escalation of oral corticosteroid is recommended 7 . Based on Joly et al study, RTX maintenance therapy every 6 months is recommended in subgroups with baseline PDAI ≥45 or patients with persistent anti‐DSG1 antibody values of 20 IU/ml or higher and/or anti‐DSG3 antibody values of 130 IU/ml or higher at month 3 36 .…”

Efficacy and Safety Data for Rituximab (Anti-CD20) in the Treatment of Pemphigus Vulgaris: A Retrospective, Single-center Study

“…In the treatment guideline published in 2020, rituximab alone or in combination with steroids is recommended as the first-line treatment for mild-tomoderate/severe pemphigus. In case of rituximab contraindication, it is recommended to use a systemic steroid alone or in combination with an appropriate immunosuppressive agent (azathioprine 1−2.5 mg/kg/day or mycophenolate mofetil 2 g/day or mycophenolate sodium 1,440 mg/day) (11).…”

Dermato‐venereology in the year of coronavirus – Hot topics in research and patient care

“…Management of anogenital warts 29 Management of pemphigus vulgaris and foliaceus 30 Use of extracorporeal photopheresis 31 …”