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Patients with PBP tend to be in their sixth or early seventh decade of life and, on average, the duration of disease is 1 year. The most common presentation of disease is urticaria-like plaques.

IgG4 is the initial and predominant anti-BMZ antibody subclass detected in PBP. Demonstration of linear C5-9 at the BMZ enhances the early diagnosis of PBP. Predominance of IgG4 and the initial presence of IgG4 on skin lesions as well as the presence of only IgG4 subclass anti-BMZ antibody suggest that IgG4 subclass antibody could be the initial immunologic event encountered in patients with PBP.