LINEAR IGA BULLOUS DERMATOSIS WITH AUTOANTIBODIES TO A 290-KD ANTIGENOF ANCHORING FIBRILS

We describe a patient with a papulovesicular eruption associated with scarring and severe mucosal lesions that led to blindness. Direct immu nofluorescence showed linear IgA deposits at the dermoepidermal juncti on. Indirect immunofluorescence microscopy showed that the patient's s erum reacted with the dermal side of salt-split skin. Direct immunoele ctron microscopy showed the IgA deposits to be associated with anchori ng fibrils, whereas with Western blot analysis the patient's serum rea cted with a 290 kd dermal antigen. On the basis of these findings, we suggest that our case may represent a form of IgA-mediated epidermolys is bullosa acquisita.