Rare autoimmune blistering disorders

Abstract

The spectrum of autoimmune blistering disorders continues to evolve as previous associations become new entities, and these entities demonstrate distinct clinical, histologic, and immunohistochemical characteristics. The rare autoimmune blistering disorders present both diagnostic and therapeutic challenges to clinicians. Diagnostically, there can be overlapping features between the rare and more common autoimmune diseases. From a therapeutic standpoint, there is a general lack of studies that demonstrate treatment efficacy and outcomes in these entities leading to clinical practice gaps. This chapter will review the clinical and histological features of lichen planus pemphigoides (LPP), bullous lichen planus (BLP), bullous systemic lupus erythematosus (SLE), IgA pemphigus, and subcorneal pustular dermatosis (SPD), and provide an evidence-based review of the treatment options reported in the literature.