Burden of Disease of Bullous Pemphigoid: A Targeted Literature Review

Bullous pemphigoid (BP) is a rare, chronic, autoimmune, blistering disease characterized by heterogenous lesions including the formation of fluid-filled blisters on frequently flexed areas of the skin and involvement of the mucosa in 10-25% of patients. BP mainly affects older patients and is associated with increased morbidity and mortality. The aim of the targeted literature review was to assess the clinical, humanistic, and economic burden associated with BP.

Targeted searches were conducted in Embase and MEDLINE for studies published in English up to November 2022 that reported burden of illness of BP. A total of 42 publications on the clinical (n = 37), humanistic (n = 6), and economic (n = 8) burden of BP were identified. BP was associated with a high mortality rate (27-72%) and an increased risk of death compared to matched controls. The majority of patients had at least one comorbidity, including hypertension, diabetes mellitus, stroke, and neurological disorders, and showed severe symptoms such as itching and pain. BP was associated with a negative impact on BP- and pruritus-related quality of life and mental health, with 31% and 38% of patients with BP experiencing depression and anxiety in the "borderline/abnormal" range, respectively. BP was further associated with high economic burden, reflected by increased healthcare resource utilization, such as longer hospital stays (7.3 days in patients with BP vs. 4.7 days in those without BP), and direct disease-related costs.

This review provides a detailed assessment of the evidence base for BP, characterizing key aspects of the disease burden including clinical, humanistic, and economic burden. The results emphasize that this rare disease is associated with a substantial burden to patients and have implications for clinical practice in the management of BP.