Skip Navigation



NDT Advance Access published online on March 26, 2007
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfm103
This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow All Versions of this Article:
22/7/2063    most recent
gfm103v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Gwinner, W.
Right arrow Articles by Haller, H.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Gwinner, W.
Right arrow Articles by Haller, H.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. for Permissions, please email: journals.permissions@oxfordjournals.org

Scleroderma-like acute renal crisis in a patient with scleromyxedema

Wilfried Gwinner1, Uta Erdbruegger1, Michael Mengel2, Carsten Hafer1, Jens Kittner3, Torsten Witte3, Bernward Voelker4 and Hermann Haller1

1Division of Nephrology, Department of Internal Medicine, 2Department of Pathology, 3Division of Immunology, Department of Internal Medicine and 4Department of Dermatology, Medical School Hannover, Hannover, Germany

Correspondence and offprint requests to: Wilfried Gwinner, Department of Nephrology, Medical School Hannover Carl-Neuberg-Str. 1, 30625 Hannover, Germany. Email: wgwinner@t-online.de

Keywords: acute renal failure; central nervous system disease; intravenons immunoglobulins; mucinosis; scleroderma; thrombotic microcangiopathy

The first 150 words of the full text of this article appear below.



   Introduction
 
Scleromyxedema is a rare connective tissue disorder, characterized by generalized skin induration with waxy yellow–red papules. Histological features include cutaneous mucin deposition in the superficial dermis, specifically hyaluronic acid, and fibroblast proliferation. Most patients with this disorder present with monoclonal paraproteinaemia, mainly IgG lambda. Oesophageal abnormalities with dysphagia, muscle weakness, dyspnoea with abnormal diffusing capacity, restrictive lung disease and cor pulmonale, pericardial effusions, central nervous system abnormalities and neuropathies, arthralgias and Raynaud's phenomenon have been observed, and may be associated with significant morbidity and mortality [1–3]. In post-mortem studies, interstitial and vascular mucin deposition in inner organs has been found inconsistently [2,4]. Renal disease is rare in patients with scleromyxedema. In this article, we report a middle-aged female with scleromyxedema and a scleroderma-like acute renal crisis.



   Case
 
A 48-year old female was admitted with dyspnoea at rest, increasing bilateral leg oedema and general weakness. In . . . [Full Text of this Article]



   Discussion
 

Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 BMJ Case ReportsHome page
N. M. E. Hossainy and M. M. E. Fatatry
Scleromyexdema?
BMJ Case Reports, June 1, 2009; 2009(jun01_1): bcr0820080626 - bcr0820080626.
[Full Text]