Retrospective Study on Autoimmune Blistering Disease in Paediatric Patients

Autoimmune blistering diseases (AIBDs) are rare in children and their prevalence in Singapore is unclear. We aimed to investigate the clinical and immunopathologic characteristics of children diagnosed with AIBDs in Singapore.

The clinical and histology databases at the National Skin Centre in Singapore were searched to identify patients younger than 18 years old diagnosed with an AIBD from January 1, 1998, through December 31, 2012. Patient demographic characteristics, presentation, triggers, investigations, treatments, and disease course were analyzed.

Twelve patients with AIBDs were identified; five (41.7%) had linear immunoglobulin A disease (LAD); two (16.7%) each had pemphigus vulgaris (PV), bullous pemphigoid, and bullous systemic lupus erythematosus; and one (8.3%) had pemphigus foliaceus. Four (33.3%) were female and eight (66.7%) male. The mean age of onset was 8.7 years (range 2-17 years). Most patients were treated with steroids and adjuvant immunosuppressants. Intravenous rituximab was used effectively in a patient with recalcitrant PV. The mean follow-up was 2.35 years (range 0.17-7.33 years). As of the last follow-up, four (33.3%) patients were in complete remission off therapy, two (16.7%) were in complete remission on therapy, four (33.3%) were in partial remission on therapy, and two (16.7%) were lost to follow-up.

Consistent with the existing literature, our study shows that LAD is the most common cause of AIBDs in children. Although common in the West, dermatitis herpetiformis was not identified in the current study. Intravenous rituximab may be considered in recalcitrant childhood PV, but vigilant monitoring for side effects is crucial. Immunohistopathologic evaluation is important and repeat biopsies may be of value in patients with atypical disease courses.