“…A recent study utilizing the PNP model mouse suggests that the fatal pulmonary symptoms in patients are most likely due to ectopic expression of Dsg3 and other epidermal antigens (Hata, et al, 2013). Autoantibodies in PNP are directed against multiple desmosomal proteins, including Dsgs, Dscs, desmoplakin and plakophilins (Futei, et al, 2003, Gallo, et al, 2014, Hashimoto, et al, 1995a, Lambert, et al, 2010, Seishima, et al, 2004). Similar to other forms of pemphigus, blistering in this disease can be attributed to autoantibodies directed against Dsgs (Amagai, et al, 1998, Nishifuji, et al, 2007, Saleh, et al, 2012), particularly the Dsg3 EC2-3 domains (Saleh, et al, 2012).…”