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

Jefferson Institute of Molecular Medicine and Division of Connective Tissue Diseases, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, PA, USA.
Journal of clinical rheumatology: practical reports on rheumatic & musculoskeletal diseases (Impact Factor: 1.25). 08/2011; 17(6):318-22. DOI: 10.1097/RHU.0b013e31822be61e
Source: PubMed

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.

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