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NDT Advance Access originally published online on July 5, 2007
Nephrology Dialysis Transplantation 2007 22(10):3065-3069; doi:10.1093/ndt/gfm354
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org


End-stage disease of native kidneys in a patient with biopsy-proven Arndt-Gottron scleromyxoedema and recurrence in the transplanted kidney

Patrick Peeters1, Marleen Praet2, Bruno Van Vlem1, Jean-Marie Naeyaert3 and Raymond Vanholder1

1Renal Unit, University Hospital of Ghent, Belgium, 2Pathology Unit, University Hospital of Ghent, Belgium and 3Dermatology Unit, University Hospital Ghent, Belgium

Correspondence and offprint requests to: Patrick Peeters, M.D. Renal Unit, Department of Internal Medicine, University Hospital UZ Gent 0K12 IA, De Pintelaan 185 9000 Gent, Belgium. Email: p.peeters{at}ugent.be



  Abstract

End-stage renal disease-stage 5 chronic kidney disease (CKD)-of the native kidneys, related to biopsy-proven Arndt-Gottron scleromyxoedema, developed in a male patient. From 1998 until 2001, the patient was treated by haemodialysis. In June 2001, cadaveric kidney transplantation was performed. In January 2004, a kidney biopsy was performed because of deteriorating renal function revealing relapse of scleromyxoedema with typical concentric narrowing of the arterioles due to accumulation of mucopolysaccharides with severe glomerular ischaemia.

Arndt-Gottron scleromyxoedema is an as yet unsuspected cause of stage 5 CKD of the native kidneys. Moreover, the disease can relapse in the transplanted kidney, again leading to intractable transplant stage 5 CKD.

Keywords: Arndt-Gottron; scleromyxoedema; end-stage renal disease; chronic kidney disease; renal failure; ischemia; kidney transplantation; mucin deposition; skin fibrosis

See http://www.oxfordjournals.org/our_journals/ndtplus/

Received for publication: 23. 1.07
Accepted in revised form: 9. 5.07


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