Demographics and Clinical Features of Autoimmune Bullous Diseases in Sarawak General Hospital

ABSTRACT     

Background: Autoimmune bullous diseases (ABDs) are organ-specific autoimmune diseases characterized by erosions, vesicles, or bullae formations on the skin or mucous membranes. The study aimed to demonstrate demography, clinical features, co-morbidities, laboratory findings, and treatment modalities for ABDs in patients in Sarawak, Malaysia. Materials and methods: This was a retrospective cross-sectional study of patients with ABDs treated at the dermatology clinic of Sarawak General Hospital over an eight years period. Results: A total of 79 patients with ABD were included in this study. ABD consisted of bullous pemphigoid (75.9%), pemphigus vulgaris (13.9%), pemphigus foliaceous (7.6%), IgA dermatosis (1.3%) and pemphigoid gestationis (1.3%). Malay ethnicity were the most commonly affected group. Bullous pemphigoid predominantly affected the elderly and female gender. In contrast, pemphigus vulgaris were more common in younger age group with a male predilection. Clinical manifestations of bullous pemphigoid and pemphigus differed in terms of its lesion extent, location, and mucosal involvement. Bullous pemphigoid was associated with multiple co-morbidities and polypharmacy. One-third of bullous pemphigoid patients had peripheral blood eosinophilia. Corticosteroid was the mainstay treatment for ABDs. The majority of ABD patients achieved remission although pemphigus patients took a longer duration compared to pemphigoid (mean 17 months vs 4 months).  Conclusion: The study illustrated an overview of the demography and characteristics of ABDs, which facilitated a detailed understanding of the disease among primary healthcare personnel. The overall clinical outcome served as a foundation to refine treatment strategy befitting the standards of clinical care.

Keywords: Autoimmune bullous disease, Bullous pemphigoid, Demography, Immunofluorescence, Pemphigus Vulgaris.