Look for the String of Pearls: A Case of Linear Immunoglobulin A Bullous Disease in a Young Boy

Abstract Linear immunoglobulin A bullous disease (LABD) is a rare autoimmune vesicobullous disease due to immunoglobulin A autoantibodies directed against various antigens at the basement membrane of the skin. In children, LABD is usually idiopathic with a benign prognosis and typically manifests with a distinct “string of pearls” configuration. We report a 6-year-old Malay boy who presented with widespread tense vesicobullous lesions arranged in a circular pattern resembling strings of pearls, predominantly in the anogenital area. Skin biopsy and direct immunofluorescence study were consistent with findings LABD, and he was treated successfully with a combination of oral dapsone and steroids. Our case report highlights the importance of recognizing typical clinical signs of this rare condition in children and diagnostic confirmation through skin biopsy.