Chronic bullous dermatosis of childhood: immunopathologic studies

Chronic bullous dermatosis of childhood (CBDC) is a distinctive subepidermal blistering disorder that characteristically involves the lower face, neck, lower trunk, and diaper area. Nearly all affected patients have a continuous linear band of IgA at the dermoepidermal junction by direct immunofluorescence and many have circulating IgA antibasement membrane antibodies. Few ultrastructural studies have been performed to determine the location of these IgA autoantibodies. We examined an infant with the typical clinical, histopathologic, and immunopathologic features of CBDC in whom a circulating, lamina lucida-reactive IgA autoantibody was identified with a new, more sensitive, split-skin indirect immunofluorescence technique.