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Scleroderma Renal Crisis-Like Acute Renal Failure Associated With Mucopolysaccharide Accumulation in Renal Vessels in a Patient With Scleromyxedema

Lee, Young H. MD; Sahu, Joya MD; O'Brien, Marie S. DO; D'Agati, Vivette D. MD; Jimenez, Sergio A. MD

JCR: Journal of Clinical Rheumatology: September 2011 - Volume 17 - Issue 6 - p 318-322
doi: 10.1097/RHU.0b013e31822be61e
Case Reports

Scleromyxedema is a systemic disease characterized by lichenoid papules, nodules, and plaques on the skin and often diffuse skin induration resembling the cutaneous involvement of systemic sclerosis. The systemic involvement affects the musculoskeletal, pulmonary, cardiovascular, gastrointestinal, and central nervous systems, and the disorder is commonly associated with a paraproteinemia. Involvement of the kidney is rare and not considered a feature of the disease. Here, we describe an unusual case of scleromyxedema complicated by the development of scleroderma renal crisis-like acute renal failure with a marked intimal deposition of mucin, mucopolysaccharides, and hyaluronic acid in the intrarenal vessels.

From the *Jefferson Institute of Molecular Medicine and Division of Connective Tissue Diseases, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, PA; †Department of Arthritis and Rheumatology, Lehigh Valley Physician Group, Allentown, PA; and ‡Department of Pathology, College of Physicians and Surgeons, Columbia University, New York, NY.

Dr Jimenez is supported by National Institutes of Health grant 5R01 AR019161.

The authors declare no conflicts of interest.

Correspondence: Sergio A. Jimenez, MD, 233 S 10th St, Suite 509 BLSB, Philadelphia, PA 19107. E-mail:

Copyright © 2011 Wolters Kluwer Health, Inc. All rights reserved.