title: Superior vena cava obstruction associated with nephrogenic systemic fibrosis.
abstract: A 56-year-old woman with hypertension-induced end stage renal disease presented with skin thickening and mottled discoloration. Cutaneous biopsy showed increased dermal fibroblasts embedded in fibromyxoid stroma with scattered perivascular and interstitial mononuclear cells. Immunohistochemistry revealed prominent CD34+ dendritic cells in septal spaces, consistent with Nephrogenic Systemic Fibrosis (NSF). Seven years and two years prior she had received a gadolinium-based contrast agent (GBCA). She died due to NSF. Gross autopsy revealed a thickened and stenotic superior vena cava (SVC). Extensive fibrosis of the SVC, dermis, and subcutaneous tissue was noted, together with hyalinized collagen fibers within the muscular wall of the intestines and dura mater. These findings support the importance of skin changes in the recognition of life threatening extracutaneous tissue involvement in NSF.
authors.forename: Kassandra E
authors.initials: KE
authors.lastname: Holzem
authors.forename: Beatrice
authors.initials: B
authors.lastname: Nardone
authors.forename: Jon W
authors.initials: JW
authors.lastname: Lomasney
authors.forename: Pedram
authors.initials: P
authors.lastname: Yazdan
authors.forename: Pedram
authors.initials: P
authors.lastname: Gerami
authors.forename: Dennis P
authors.initials: DP
authors.lastname: West
authors.forename: Anne E
authors.initials: AE
authors.lastname: Laumann
mesh_terms: D003287:Contrast Media / Q000008:administration & dosage / Q000009:adverse effects; D017809:Fatal Outcome; D005260:Female; D005682:Gadolinium / Q000008:administration & dosage / Q000009:adverse effects; D006801:Humans; D007150:Immunohistochemistry; D007676:Kidney Failure, Chronic; D008875:Middle Aged; D054989:Nephrogenic Fibrosing Dermopathy / Q000150:complications* / Q000209:etiology / Q000503:physiopathology; D013479:Superior Vena Cava Syndrome / Q000209:etiology* / Q000503:physiopathology
chemical_list: D003287:Contrast Media; D005682:Gadolinium