THE USE OF SKIN SUBSTRATES DEFICIENT IN BASEMENT-MEMBRANE MOLECULES FOR THE DIAGNOSIS OF SUBEPIDERMAL AUTOIMMUNE BULLOUS DISEASE

A case is presented of subepidermal, autoimmune bullous disease in whi ch the initial examinations suggested the combination of epidermolysis bullosa acquisita and bullous pemphigoid. The diagnosis of epidermoly sis bullosa acquisita was made by indirect immunofluorescence microsco py: the patient's serum bound to normal skin substrate but not to type VII collagen-deficient skin substrate derived from a patient with mut ilating dystrophic epidermolysis bullosa. The use of skin substrates d eficient in basement membrane molecules also excluded the presence of concomitant, circulating bullous pemphigoid autoantibodies in our pati ent. The diagnosis of epidermolysis bullosa acquisita was confirmed by split mapping, fluorescence overlay antigen mapping and Western blot.