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.