Nephrogenic systemic fibrosis associated with stromal and vascular calcification, report of two cases
- 17 September 2009
- journal article
- case report
- Published by Wiley in Journal of Cutaneous Pathology
- Vol. 36 (s1), 31-34
- https://doi.org/10.1111/j.1600-0560.2008.01205.x
Abstract
Nephrogenic systemic fibrosis (NSF) is a systemic fibrosing disorder characterized by the development of large indurated plaques on the skin, primarily in patients with end‐stage renal disease (ESRD). Skin biopsy reveals an increased frequency of CD34+ and Factor XIIa+ cells. Microscopic calcification has been reported in the skin biopsies of rare cases of NSF. The etiology and significance of this calcification is not clear. We present two cases of typical NSF, for which marked stromal and vascular calcification was identified on skin biopsy. In both cases, the calcification was within typical NSF lesions and was intimately associated with aggregates of CD34+, Factor XIIIa+ spindle and stellate cells. This particular pattern of calcification strongly argues against either nonspecific dystrophic calcification or coincidental metastatic calcification. These findings suggest that calcification may be intrinsic to the pathophysiology of NSF, at least in a subset of NSF patients. In addition, the vascular calcification involving small arteries and arterioles in these two cases morphologically resembled calciphylaxis, which is another poorly understood complication of ESRD. This resemblance raises the possibility that NSF may predispose to or develop in the vicinity of indolent lesions of early‐stage calciphylaxis. We propose that skin biopsies performed as part of a diagnostic workup for suspected NSF should preferably include the deep dermis and subcutis, in order to assess possible vascular calcification.Keywords
This publication has 12 references indexed in Scilit:
- The Role of the Hospital Dermatologist in the Diagnosis and Treatment of Calciphylaxis and Nephrogenic Systemic FibrosisSeminars in Cutaneous Medicine and Surgery, 2007
- Nephrogenic systemic fibrosis after exposure to gadolinium in patients with renal failureNephrology Dialysis Transplantation, 2007
- Gadolinium deposition in nephrogenic fibrosing dermopathyJournal of the American Academy of Dermatology, 2007
- Nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum‐like changesJournal of Cutaneous Pathology, 2006
- Calciphylaxis – a topical overviewJournal of the European Academy of Dermatology and Venereology, 2006
- Nephrogenic fibrosing dermopathyJournal of the American Academy of Dermatology, 2005
- Calciphylaxis and metastatic calcification associated with nephrogenic fibrosing dermopathyJournal of Cutaneous Pathology, 2004
- Nephrogenic Fibrosing Dermopathy With Systemic InvolvementArchives of Dermatology, 2003
- Nephrogenic Fibrosing DermopathyThe American Journal of Dermatopathology, 2001
- Scleromyxoedema-like cutaneous diseases in renal-dialysis patientsThe Lancet, 2000