Nephro‐urological complications of epidermolysis bullosa in paediatric patients

Summary A small but important proportion of patients with epidermolysis bullosa (EB) may develop significant renal and urological complications which can have a major impact on their morbidity and mortality. During the last 10 years, five of a large group of children with EB under our care, with either dystrophic or junctional types of disease, experienced major nephro‐urological complications. Two patients with recessive dystrophic EB (REDB) developed macroscopic haematuria ‐ one had renal failure and underwent a renal biopsy showing IgA nephropathy. A third patient with RDEB also developed renal failure and his biopsy demonstrated postinfectious glomerulonephritis/type III membranoproliferative (mesangiocapillary) glomerulonephritis. Both patients with renal failure underwent peritoneal dialysis. Two patients with junctional EB developed obstructive uropathies, which required bladder reconstruction and the fashioning of a Mitrofanoff channel in one.