Superior vena cava obstruction associated with Nephrogenic Systemic Fibrosis

Kassandra E. Holzem, Beatrice Nardone, Jon W. Lomasney, Pedram Yazdan, Pedram Gerami, Dennis P. West, Anne E. Laumann*

*Corresponding author for this work

Research output: Contribution to journalArticle

2 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)615-618
Number of pages4
JournalJournal of Drugs in Dermatology
Volume13
Issue number5
StatePublished - Jan 1 2014

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ASJC Scopus subject areas

  • Medicine(all)
  • Dermatology

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