Mixed immunobullous disease of childhood: a good response to antimicrobials

Background Immunobullous diseases are uncommon in childhood. In contrast to adults, the most commonly seen is IgA-mediated chronic bullous disease of childhood (CBDC), while IgG-mediated bullous pemphigoid (BP), cicatricial p emphigoid (CP) and epidermolysis bullosa acquisita (EBA) are rare, We have demonstrated both IgG and IgA autoantibodies to basement membrane zone targ et antigens in eight children with 'mixed immunobullous disease of childhoo d'. Objectives To elucidate whether a dual antibody response makes these patien ts distinct regarding their presentation, immunopathology, course and progn osis. Methods We compared the eight children showing the double antibody response with 62 children with CBDC, BP, CP and EBA in whom only one antibody isoty pe was demonstrated. Clinical information at presentation, clinical course and response to treatment were recorded, and immunoblotting and direct and indirect immunofluorescence (IF) were performed, Results Six of the eight patients presented with clinical features of CBDC, In two others, it was uncertain whether they had CBDC or BP. Seven of the eight demonstrated a dual antibody response on indirect IF and three on dir ect IF, Immunoblotting revealed a variety of epidermal and dermal target; a ntigens (BP230, BP180, 97-kDa protein and laminin 5), Five of the eight res ponded well to dapsone, two to sulphonamides, and one to systemic erythromy cin alone, The clinical course was not protracted. Five are in remission 1- 4 years following treatment, and three still have active disease suppressed by treatment after 6 months-2 years. Conclusions Although we do not know why these children have 'mixed immunobu llous disease' (the dual antibody response), our results indicate that the presence of IgA is associated with a good response to treatment with antimi crobials (dapsone, sulphonamides, erythromycin), and the clinical course is no more protracted than that found in children with a single antibody resp onse.