Nephrogenic systemic fibrosis (NSF) is an acquired condition that occurs in the setting of chronic kidney disease (CKD), and it eventuates as fibrosis of the skin, soft tissues, and sometimes the viscera. For nearly a decade, the cause of NSF remained largely elusive. Only recently have the combined efforts of multiple investigators shed light on key associations and possible reasons for the development of disease. First described by Cowper et al1 in 2000 (using tissue dating from 1997), NSF was distinguished clinically by thickening and hardening of the skin and histologically by increased dermal spindle cells, with resultant fibrosis and mucin deposition. While in some ways NSF resembles scleromyxoedema, patients lack the characteristic paraproteinemia and other stigmata of that disease.
High WA. Estimates of Risk, Empirical Treatment Observations, and Unexpected Laboratory Findings Reveal the Complexity of Nephrogenic Systemic Fibrosis. Arch Dermatol. 2009;145(10):1178-1182. doi:10.1001/archdermatol.2009.227