title: Scleroderma renal crisis-like acute renal failure associated with mucopolysaccharide accumulation in renal vessels in a patient with scleromyxedema.
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.
authors.affiliation: Jefferson Institute of Molecular Medicine and Division of Connective Tissue Diseases, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, PA, USA.
authors.forename: Young H
authors.initials: YH
authors.lastname: Lee
authors.forename: Joya
authors.initials: J
authors.lastname: Sahu
authors.forename: Marie S
authors.initials: MS
authors.lastname: O'Brien
authors.forename: Vivette D
authors.initials: VD
authors.lastname: D'Agati
authors.forename: Sergio A
authors.initials: SA
authors.lastname: Jimenez
mesh_terms: D058186:Acute Kidney Injury / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism*; D001706:Biopsy; D017809:Fatal Outcome; D005260:Female; D006025:Glycosaminoglycans / Q000378:metabolism*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D008875:Middle Aged; D012077:Renal Artery / Q000378:metabolism*; D012082:Renal Veins / Q000378:metabolism*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D053718:Scleromyxedema / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism
chemical_list: D006025:Glycosaminoglycans; D016756:Immunoglobulins, Intravenous