Autoimmune bullous dermatoses

Abstract

SummaryAutoimmune bullous dermatoses (AIBD) are a heterogeneous group of about a dozen diseases characterized clinically by erosions and blisters and immunopathologically by autoantibodies against structural proteins of the skin or transglutaminase 2/3. The diagnosis of AIBD has made tremendous progress in the last decade due to the availability of standardized serological assays that, knowing the clinical picture, allow the diagnosis in the vast majority of patients. The development of various in vitro and in vivo models of the most common AIBD, namely, bullous pemphigoid, pemphigus vulgaris, mucous membrane pemphigoid, and the rare epidermolysis bullosa acquisita, allows identification of key molecules and inflammatory pathways as well as preclinical evaluation of the effect of new anti‐inflammatory agents. The approval of rituximab for moderate and severe pemphigus vulgaris and the development of national and international guidelines for the most common AIBD have considerably advanced the care of these patients. Nevertheless, the limited therapeutic armamentarium is the main challenge for the management of AIBD. Several phase II and III randomized controlled clinical trials provide hope for new, effective, and safe therapeutic options in the coming years. This review summarizes the epidemiology, clinic, diagnosis, pathophysiology, and therapy of AIBD and gives an outlook on both current diagnostic and therapeutic needs as well as future developments.

Successful treatment of checkpoint inhibitor‐associated bullous pemphigoid with dupilumab in a patient with angiosarcoma

“…BP180-NC16A was elevated in ELISA. This confirmed the diagnosis of BP, 6 which we evaluated in association with the ICI. ICI therapy was therefore paused and oral steroid pulse therapy with prednisolone 80 mg (1 mg/kg body weight) was initiated.…”

Epidermolysis bullosa acquisita induced by atezolizumab

“…Epidermolysis bullosa acquisita (EBA) is a rare, acquired, autoimmune subepidermal bullous disease 1 . EBA is associated with autoantibodies against type VII collagen, which is the major component of the anchoring fibrils of the dermal‐epidermal junction 2 .…”

Epidermolysis bullosa acquisita ausgelöst durch Atezolizumab

“…Epidermolysis bullosa acquisita (EBA) ist eine seltene, erworbene, autoimmune, subepidermale bullöse Erkrankung 1 . Epidermolysis bullosa acquisita geht mit Autoantikörpern gegen Kollagen Typ VII einher, dem Hauptbestandteil der Verankerungsfibrillen der dermoepidermalen Junktionszone 2 .…”