ABSTRACT

A 32-yr-old man with epidermolysis bullosa presented with clinical and laboratory findings of chronic renal failure. The patient was supposed to be suffering from mesangial IgA glomerulonephritis in view of the repeated persistent macroscopic episodes of hematuria and raised serum IgA levels, especially polimeric IgA. Because continuous vascular access could not be established, the patient died due to uremia and sepsis. Renal complications are associated with life-threatening problems in this inherited mechanobullous disease because it is impossible to obtain a continuous vascular access or a continuous peritoneal access. The possibility of IgA nephropathy should be considered in patients with epidermolysis bullosa. They should be periodically set up screened for IgA levels and hematuria.