Clinical characteristics and treatment outcomes of linear IgA bullous dermatosis

Abstract

Linear immunoglobulin A (IgA) bullous dermatosis (LABD), also referred to as chronic bullous disease of childhood (CBDC), is characterized by the linear deposition of IgA antibodies within the dermal-epidermal junction. While dapsone is typically recommended, alternative modalities may be considered based on accessibility, severity, and prior response to therapy. This review aims to provide an updated overview of the clinical characteristics and treatment outcomes for LABD/CBDC. A systematic review was conducted using MEDLINE and Embase in adherence to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Results from 650 articles, encompassing 1,627 cases, revealed 52% of cases were male. Vesicles (49%) and bullae (47%) were the predominant morphologies occurring across multiple body locations simultaneously, such as the legs (52%), abdomen (49%), and back (49%). Vancomycin was the most reported causative medication; however, most cases were not drug-induced. Dapsone was the primary therapy for LABD/CBDC while biologics were reported with higher complete response rates. Other immunomodulators, such as IVIG and mycophenolate mofetil, were reported to have comparative responses in CBDC cases, but lower response rates in LABD cases. Colchicine and amoxicillin-clavulanate had lower response rates overall. The variety of treatment options underscores management challenges due to the variable clinical presentations and underlying causes.