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End-stage renal disease secondary to IgA nephropathy in recessive dystrophic epidermolysis bullosa: a case report.

Tammaro F, Calabrese R, Aceto G, Lospalluti L, Garofalo L, Bonifazi E, Piccolo T, Pannarale G, Penza R

Department of Biomedicine of Developing Age, U.O. "B Trambusti", University of Bari, Piazza Giulio Cesare, 11, 70124, Bari, Italy, penza@bioetaev.uniba.it.

Epidermolysis bullosa (EB) consists of a group of dominant or recessive autosomal diseases characterised by skin and mucosa fragility. The lesions leave erosions and scars that, in turn, can cause stenosis of tracheal, oesophageal, and genitourinary tract mucosae. The significantly increased survival of EB patients has determined the onset of complications never observed before, including genitourinary disorders such as hydroureteronephrosis, recurrent urinary tract infections, renal amyloidosis, IgA nephropathy and post-infectious glomerulonephritis. A 6-year-old boy diagnosed with recessive dystrophic EB Hallopeau-Siemens type (RDEB-HS) was referred to our clinic because of microhaematuria that evolved into intra-infectious macrohaematuria. Renal biopsy revealed an increase in both extracellular matrix and mesangial cells, with a focal segmental glomerulosclerosis with severe chronic tubulointerstitial damage. Immunofluorescence showed IgA mesangium deposits. Five years later, he was started on haemodialysis, because of worsening renal function. This is a rare case of a child with EB who was successfully treated with haemodialysis. The pertinent literature has been reviewed.

Published 6 December 2007 in Pediatr Nephrol, 23(1): 141-4.
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