Scleroderma renal crisis-like acute renal failure associated with mucopolysaccharide accumulation in renal vessels in a patient with scleromyxedema.

Authors

Young H Lee, Thomas Jefferson University
Joya Sahu, Thomas Jefferson UniversityFollow
Marie S O'Brien, Thomas Jefferson University
Vivette D D'Agati, Thomas Jefferson University
Sergio A. Jimenez, Thomas Jefferson UniversityFollow

Document Type

Article

Publication Date

9-1-2011

Comments

This article has been peer reviewed. It is the authors' final version prior to publication in Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases

Volume 17, Issue 6, September 2011, Pages 318-322.

The published version is available at DOI: 10.1097/RHU.0b013e31822be61e. Copyright © Lippincott, Williams and Wilkins

Abstract

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.

Recommended Citation

Lee, Young H; Sahu, Joya; O'Brien, Marie S; D'Agati, Vivette D; and Jimenez, Sergio A., "Scleroderma renal crisis-like acute renal failure associated with mucopolysaccharide accumulation in renal vessels in a patient with scleromyxedema." (2011). Scleroderma Center Faculty Papers. Paper 5.
https://jdc.jefferson.edu/sclerodermafp/5

PubMed ID

21869708