CHRONIC BULLOUS DISEASE OF CHILDHOOD AND LINEAR IGA DISEASE OF ADULTSARE IGA1-MEDIATED DISEASES

Linear IgA disease is characterized by the presence of linear IgA depo sits at the basement membrane zone of the skin, and in some cases by c irculating basement membrane zone antibodies. The disease occurs in bo th adults and children, and is designated adult linear IgA disease in the former and chronic bullous disease of childhood in the latter. The subclass distribution of the circulating and bound basement membrane zone antibodies was studied in 32 children and eight adults. The resul ts were compared with live dermatitis herpetiformis patients and five normal controls. The circulating antibodies (39 patients) and the cuta neous deposits (39 patients) were IgA1 in all 40 patients with linear IgA disease. The cutaneous deposits in dermatitis herpetiformis were a lso all IgA1, and no circulating antibodies were detected. The control s were all negative. This large series of children and adults with lin ear IgA disease demonstrates that the circulating and cutaneous baseme nt membrane zone deposits are all IgA1, and suggests that linear IgA d isease is an IgA1-mediated disease.