Scleroderma renal crisis-like acute renal failure associated with mucopolysaccharide accumulation in renal vessels in a patient with scleromyxedema.
Publication/Presentation Date
9-1-2011
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.
Volume
17
Issue
6
First Page
318
Last Page
322
ISSN
1536-7355
Published In/Presented At
Lee, Y. H., Sahu, J., O'Brien, M. S., D'Agati, V. D., & Jimenez, S. A. (2011). Scleroderma renal crisis-like acute renal failure associated with mucopolysaccharide accumulation in renal vessels in a patient with scleromyxedema. Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases, 17(6), 318–322. https://doi.org/10.1097/RHU.0b013e31822be61e
Disciplines
Medicine and Health Sciences
PubMedID
21869708
Department(s)
Department of Medicine
Document Type
Article