Factors associated with the activity and severity of bullous pemphigoid: a review

Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease. It usually affects people older than 70 years of age. The two main autoantigens are BP180 and BP230, both of which are components of hemidesmosomes. Immunoglobulin (Ig)G and IgE autoantibodies to BP180 detected by the enzyme-linked immunoassay (ELISA) show close associations with the activity and severity of BP. In addition, inflammatory cells (eosinophils, neutrophils and mast cells) and cytokines (e.g. interleukins and CC chemokine ligands) play an important part in the pathogenesis, activity and severity of BP. We summarized the potential contribution of each factor postulated to be associated with the activity and severity of BP, and provide guidance for clinicians to pay timely and close attention to such parameters. This review may also promote the development of novel therapies for BP.Key MessagesBullous Pemphigoid Disease Area Index (BPDAI) is a scoring system which can reflect the extent of clinical involvement of BP patients.The titres of IgE autoantibodies and IgG autoantibodies against the NC16A domain of BP180 are closely correlated with the activity and severity of BP.Many inflammatory cells and molecules, such as eosinophils and interleukins, can also reflect the activity and severity of BP.