Autoimmune Bullous Diseases in Ipoh, Malaysia: A 5-year Retrospective Study

ABSTRACT

Background Autoimmune bullous diseases (ABD) represent a group of chronic blistering dermatoses in which management is often challenging. Epidemiologic data on these diseases in Malaysia has been limited. Objectives Our purpose was to study the spectrum of the various ABD presented to the Department of Dermatology, Ipoh Hospital, and to determine the clinico-epidemiological pattern of the 2 main ABD, namely pemphigus and bullous pemphigoid. Methodology We performed a retrospective review of records for all patients who were diagnosed with ABD confirmed by histopathology and direct immunofluorescence test in this centre between 2001 and 2005. The data were analyzed with regard to age, sex, ethnicity, subtypes of ABD, treatment provided and outcome. Results There were a total of 79 cases of ABD presented to us during this period. Bullous pemphigoid was observed to be the commonest (60.8%) followed by the pemphigus group (36.7%) with the mean incidence of 0.45/100,000/year and 0.28/100,000/year respectively.44% of patients were of ethnic Chinese origin. There was an overall female preponderance. The mean age of presentation was 65.5 years for bullous pemphigoid and 55 years for pemphigus group. The mean duration of disease before presentation was 1.6 months for bullous pemphigoid and 6.3 months for pemphigus. Various combinations of immunosuppressive agents were used to treat the patients. 48% of bullous pemphigoid cases were controlled with prednisolone alone while 67.9% of pemphigus group required at least 2 immunosuppressive agents to achieve disease control. Conclusion In our study population, bullous pemphigoid was more frequently seen than pemphigus.