An In-Depth Understanding of The Pathophysiology and Therapeutic Approaches to Chronic Bullous Disease of Childhood (CBDC)

Chronic bullous disease of childhood (CBDC), also known as childhood linear IgA bullous dermatosis, is a rare autoimmune blistering disorder characterized by subepidermal blister formation and linear immunoglobulin A (IgA) deposition along the basement membrane zone. Its heterogeneous clinical presentation and similarities to other autoimmune blistering disorders pose diagnostic and therapeutic challenges. This study aimed to synthesize current evidence regarding the pathophysiology, clinical manifestations, diagnostic approaches, and therapeutic management of CBDC. A descriptive literature review was conducted using relevant original research articles, case reports, review articles, and clinical guidelines. The literature was organized thematically and comparatively evaluated to identify consistent findings, variations in clinical presentation, and limitations in the available evidence. The reviewed studies identified IgA autoantibodies targeting components of the dermal–epidermal junction, particularly BP180 (type XVII collagen), as important contributors to inflammatory responses and subepidermal blister formation. Clinical manifestations included tense vesicles and bullae, frequently exhibiting a characteristic "string of pearls" arrangement, with variable mucosal involvement. Direct immunofluorescence (DIF) examination of perilesional skin remained essential for diagnostic confirmation because clinical and histopathological features could overlap with those of other blistering disorders. Dapsone was identified as a first-line treatment, while systemic corticosteroids and alternative therapeutic agents were considered according to disease severity, treatment response, and tolerability. However, the predominance of observational evidence limited definitive comparisons of treatment effectiveness and safety. In conclusion, CBDC requires a comprehensive diagnostic approach and individualized therapeutic management. Further prospective pediatric studies are needed to strengthen the evidence regarding treatment efficacy, safety, recurrence, and long-term clinical outcomes in affected children.