Linear IgA disease (chronic bullous dermatosis of childhood):

Linear IgA disease is a subepidermal blistering disorder of adults and children. Pathogenic IgA autoantibodies can bind to a variety of basement membrane proteins including ladinin, BP 180 antigen and laminin 5 (see Fig. 23.27). It is the most common immunobullous disease seen in children. Rarely it is drug induced by vancomycin.
Clinical features
Linear IgA disease can present with circular clusters of large blisters, a pemphigoid type of blistering or a dermatitis herpetiformis picture. Mucosal involvement of the mouth, vulva and eyes is not uncommon and can cause scarring. Direct IMF studies of skin show linear IgA deposition along the basement membrane.
Treatment
This is with oral dapsone (50-200 mg daily) or sulphonamides. Occasionally immunosuppression is needed. Many patients show spontaneous resolution after 3-6 years.