Recalcitrant case of linear IgA bullous dermatosis in a child

Linear IgA bullous disease (LABD) is a rare autoimmune subepidermal blistering disorder characterized by immunoglobulin class A (IgA) deposits in the basement membrane of the skin. It affects both adults and children. The childhood variant is also known as chronic bullous dermatosis of childhood (CBDC), and unlike in adults, it has characteristic clinical findings termed as “string of pearls” or “crown of jewel” appearance of blisters. Most of the patients respond well to dapsone and corticosteroids. We report a case of a patient with CBDC who had a poor response to the above conventional therapy and had a favorable response to intravenous immunoglobulin (IVIg).