Immune cells in pemphigus vulgaris and bullous Pemphigoid: From pathogenic roles to targeting therapies

Abstract

Pemphigus vulgaris (PV) and bullous pemphigoid (BP) are two major subtypes of autoimmune bullous diseases (AIBD), characterized by blisters and erosions of skin and/or mucous membranes with dysregulated immune activity. Current literature established that T and B cells are the main executors of PV and BP. Emerging evidence revealed that macrophages and related cytokines also contribute to these diseases. While the role of lymphocytes on PV and BP is well established, the definitive functions of macrophages in disease progression are not fully understood. Furthermore, current status of clinical trials targeting immune cells is poorly recapitulated in PV and BP. In this review, we summarized current knowledge in this rapidly advancing field, with emphasis on the individual functions of immune cells and their interactions, as well as ongoing clinical trials targeting immune cells, to provide novel insights in mechanistic understanding and clinical management of PV and BP.

Keywords: Autoimmunity; Bullous pemphigoid; Lymphocytes; Macrophage; Pemphigus vulgaris.