Juvenile Bullous Pemphigoid with Similarity Clinical Features to Chronic Bullous Disease of Childhood

Abstract

Introduction: Autoimmune bullous disease (ABD) is a specific autoimmune skin disease characterized by subepidermal vesicles and bullae. In pediatrics, there is an autoimmune bullous disease controversy with clinical clusters of jewel on Chronic bullous disease of childhood (CBDC) but dominant IgG autoantibodies. A similar clinical presentation challenges diagnosis; thus, supporting studies on direct immunofluorescence (DIF) as a specific autoantibody factor are necessary.

Case: A 10-year-old boy with clinically tense-walled blisters with or without an erythematous base is itchy all over his body, with a predominance of palmoplantar, neck, inguinal, and axillary, presented by erosions, crusts, and a partial cluster of jewels lesion with mucosal involvement. A complete blood count shows leukocytosis and eosinophilia. Hematoxylin-eosin (HE) staining revealed a subepidermal cleft with eosinophil predominance, while direct immunofluorescence (DIF) revealed IgG deposits in the basal membrana and intercellular parts of the basal stratum.

Discussion: Histopathology reveals subepidermal blisters of eosinophil dominance, and direct immunofluorescence (DIF) examination reveals IgG autoantibody deposit dominance, verifying the diagnosis of juvenile bullous pemphigoid. Given that BP180 (BPAG2) is one of the autoantigens involved, there may be similarities between the histology and clinical presentation of Bullous pemphigoid (BP) and CBDC.

Conclusion: Autoimmune bullous diseases are difficult to differentiate clinically, histological studies and direct immunofluorescence are necessary to make a diagnosis.