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Superior vena cava obstruction associated with nephrogenic systemic fibrosis. J Drugs Dermatol 2014 May;13(5):615-8

Date

05/09/2014

Pubmed ID

24809889

Scopus ID

2-s2.0-84900527752   2 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.

Author List

Holzem KE, Nardone B, Lomasney JW, Yazdan P, Gerami P, West DP, Laumann AE

Author

Kassandra E. Holzem MD Assistant Professor in the Dermatology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Contrast Media
Fatal Outcome
Female
Gadolinium
Humans
Immunohistochemistry
Kidney Failure, Chronic
Middle Aged
Nephrogenic Fibrosing Dermopathy
Superior Vena Cava Syndrome