Pathophysiology of Autoimmune Bullous Diseases: Nature Versus Nurture

Indian J Dermatol. 2017 May-Jun;62(3):262-267. doi: 10.4103/0019-5154.159620.

Abstract

Pemphigus and pemphigoid are the prototypical immunobullous diseases. Although it has been well established that they are caused by deposition of autoreactive antibodies directed against adherence proteins within the skin, the specific genetic and environmental factors leading to development of these diseases continue to be an area of investigation. Herein, we discuss several of the potential environmental triggers that may induce patients to develop immunobullous diseases including medications, viral infections, UV exposure or other radiation injury and dietary factors. In addition, the potential genetic and immunologic mechanisms contributing to the pathogenesis of pemphigus and pemphigoid will be reviewed. The multifactorial nature of these diseases contributes to their complexity and highlights the importance of a detailed personal and family history when caring for these patients.

Keywords: Autoimmune blistering diseases; desmoglein; pemphigoid; pemphigus.