Characteristics of patients with bullous pemphigoid: comparison of classic bullous pemphigoid to non-bullous pemphigoid.

Abstract

Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease. Patients occasionally present with a clinical picture of pruritus/urticaria alone for months and do not even develop blisters over time. Only few studies have investigated this subgroup of non-bullous pemphigoid (NBP). To evaluate the demographic and clinical characteristics of BP patients with or without blisters at the time of diagnosis. A retrospective study based on the medical records of 115 BP patients. Collected data included demographic characteristics, clinical presentation, treatment and response to treatment. Thirty-six patients presented with pruritus/urticaria (31.3%), and 79 presented with blisters (68.7%), with mean ages of 77.5 and 76.0, respectively, at diagnosis and an equal female:male ratio. The level of immunoglobulin E (IgE) was 4.1 times higher, and the mean blood eosinophil count was significantly increased in the pruritus/urticaria group. Remission rate at 3months and relapse rate were similar between the groups. Median follow-up period was 9months (range 3-18). Only 23% of the patients with pruritus/urticaria developed blisters. A significant number of BP patients present without blisters. We found no significant epidemiological or clinical differences from the classic BP patients aside from significantly elevated IgE and blood eosinophil levels. Similar results in larger cohort studies might be the foundation for a change in clinical protocols regarding the diagnosis and recommended treatment for the elderly presenting with pruritus/urticaria only.