Prevalence and Age Distribution of Pemphigus and Pemphigoid Diseases in Germany

Autoimmune bullous diseases (AIBDs) are characterized by autoantibodies against structural proteins of the epidermis (pemphigus vulgaris and foliaceus) and dermal-epidermal junction (bullous pemphigoid, mucous membrane pemphigoid, pemphigoid gestationis, linear IgA disease, and epidermolysis bullosa acquisita) (Schmidt and Groves, 2016). Clinically, blisters and erosions arise on skin and surface-close mucous membranes. Several reports have estimated the incidence of all AIBDs at between 13.3 and 66 cases per million people per year in Germany, the UK, Switzerland, France, Finland, Italy, and the US, with the highest incidences of bullous pemphigoid occurring in the population older than 80 years of age (150–300 cases/million people/year) and the lowest for epidermolysis bullous acquisita (0.2–0.5 cases/million people/year) (Bertram et al., 2009; Brick et al., 2014; Cozzani et al., 2001; Forsti et al., 2014; Joly et al., 2012; Langan et al., 2008; Marazza et al., 2009; reviewed in Schmidt et al., 2015).