Successful maintenance hemodialysis with permanent vascular catheter in a patient with recessive dystrophic epidermolysis bullosa hereditaria

Abstract

Recessive dystrophic epidermolysis bullosa hereditaria is characterized by generalized blistering of the skin and mucous membrane in response to slight mechanical stimulation. Although renal complications such as renal amyloidosis, IgA nephropathy and hydroureteronephrosis lead to chronic renal failure, which progresses to end stage kidney disease (ESKD), it is difficult to select either hemodialysis or peritoneal dialysis as renal replacement therapy. A 34-year-old male was diagnosed with recessive dystrophic epidermolysis bullosa hereditaria and had been treated by dermatologist. He required blood purification because of congestive heart failure derived from ESKD. We considered it difficult to introduce peritoneal dialysis due to low proteinemia as well as difficult to keep his skin clean for frequent needle puncture. Therefore, we selected maintenance hemodialysis with a permanent vascular catheter. Currently, he has been continuing this therapy and is in good clinical condition.