Linear IgA bullous disease of childhood: an experience from Kuwait

Linear IgA bullous disease of childhood is a rare autoimmune blistering disease. We report eight patients with this disease seen in our autoimmune bullous diseases clinic over a span of 12 years. They constituted 89% of the total number of those with linear IgA bullous disease of childhood seen during this period, with an age-adjusted minimum estimated incidence of 2.3 cases/million population/year. Males outnumbered females by a 1.7:1 ratio. The age at onset ranged between 10.5 months and 13 years, with a mean of 6.8 +/- 4.17 years. The majority of patients (62.5%) had moderately severe disease. Fifty percent of patients were observed to have an association with either an autoimmune disease (Crohn disease and post-streptococcal glomerulonephritis in one each) or an infection (beta-hemolytic streptococcal and hepatitis A virus infection in one each). The treatment of choice was dapsone alone or in combination with systemic steroids. Seventy-one percent of patients achieved complete remission by the end of 2 years. The study highlights the significance of systematic clinicoepidemiologic surveys from different regions.