Characteristics of newly diagnosed bullous pemphigoid patients

Abstract

Introduction Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease that mainly affects elderly individuals with estimated rates < 5 in 100,000 persons [1]. It presents with pruritus followed by urticarial plaques and tense bullae on the skin [2]. Since pruritus is a common symptom in the elderly population, BP should be always considered [3] and detailed clinical and pathophysiological examination performed in order to diagnose the proper disease. Prompt diagnosis helps to manage the disease successfully, prevent complications and improve qualityof life. Aim To analyze medical characteristics of newly diagnosed BP patients treated in the Department of Skin and Venereal Diseases, Hospital of Lithuanian University of Health Sciences Kauno klinikos. Methods Patients with newly diagnosed and previously not treated BP from October 2021 to October 2022 were included in the study. Age, gender, BP disease area index (BPDAI) activity, damage scores, pruritus, histology, direct and indirect immunofluorescence (DIF, IIF), enzyme-linked immunoassay (ELISA) were included in the obtained data. BP severity was divided into 3 groups according to BPDAI score: mild BP (BPDAI score <20), moderate BP (20≤ BPDAI score <57),severe BP (BPDAI score ≥57). Statistical analysis was performed using IBM SPSS Statistics software (v. 28.0). Descriptive statistics including frequency, median (min-max) and Chi-square test were used for assessing differences between the variables. Data differences were considered statistically significant at p<0.05.Results10 patients were included in the study (6 female, 4 male, median age 74 (61-85)). Mild, moderate and severe BP were diagnosed in 80 %, 10 % and 10 % of patients. Patients with a mild BP were younger (age <75) (p=0,03). Mucous membranes were affected in 2 male patients (20%). Pruritus was observed in all patients with median severity of 5 in VAS scale (2-8 points) and higher values in male patients (>5 points) (p=0,01). Histopathology revealed mixed infiltration with lymphocytes and eosinophils (50%) at subepidermal blisters. DIF was positive for all patients with IgG and C3 deposits at the level of basement membrane in 60% of cases. IIF from serum was performed in 60% of cases, positive results were observed for half of them. IIF was more frequently positive in subjects whose BPDAI damage score was >5 points (p=0,014). ELISA from serum was positive for 70% of patients with median anti-BP180 value of 1,31 RU/mL (0,15-10,57RU/mL) and median anti-BP230 value of 0,315 RU/mL (0,1-2,06 RU/mL). All negative ELISA patients with anti-BP180 values <1,00 (RU/mL) were also negative on IIF. ELISA results of antiBP180 were lower in mild BP patients (<5,00 RU/mL) (p=0,031). Conclusions Majority of newly diagnosed patients were women with a mild BP. BP course was more severe in older patients with more intensive pruritus, higher serum anti-BP180 levels and positive IIF results. Similar results in larger studies could be a foundation to establish BP for elderly patients presenting with pruritus at an early stage and improve outcomes of BP.