Autoimmune bullous diseases: Clinical analysis, therapeutic response, and mortality in a university medical centre in Chile Enfermedades ampollares autoinmunes: caracterización clínica, respuesta terapéutica y mortalidad en un centro universitario de Chil

Abstract

Introduction: Autoimmune Bullous Diseases (ABDs) are acquired dermatoses characterised by blisters on the skin and mucous membranes. The aim of this study is to describe the epidemiology, clinical features, therapeutic response, and mortality rates in patients with ABDs treated at a university medical centre in Chile. Method: Retrospective cohort study was conducted over a 12 year period (2005-2017) of patients with ABDs confirmed by histology and direct immunofluorescence at a university medical centre in Chile. Results: Of a total of 89 patients, 50.9% were diagnosed with Bullous Pemphigoid (BP), 24.7% with Pemphigus Vulgaris (PV), and 24.7% with other ABD. The mean age in BP was 72.2 years, and 46.3 years in PV. Blisters appeared in 93.2% of BP, and mucous compromise appeared in 77.3% of PV. In BP, the most used treatment was prednisone and topical corticosteroids (TC), while in PV it was prednisone with azathioprine and TC. Half (50%) of BP achieved remission after 2 months of treatment, while in PV it was achieved after 5 months. The one-year survival rate in patients with BP was 88.7%, and 96.3% in PV. Conclusions: BP occurred at an older age, with limb and trunk blisters, whereas PV occurred in middle-aged patients, with significant mucosal involvement. Patients with PV required greater immunosuppression, reaching remission later than in BP. However, survival was lower in patients with BP. This study, the first in Chile, allowed ABDs to be characterised in Latin America.