Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis

Report of a new case with literature review

Sumanth Reddy Daram, Cherise M. Cortese, Bahar Bastani

Research output: Contribution to journalArticle

129 Citations (Scopus)

Abstract

•Nephrogenic fibrosing dermopathy (NFD) is a fibrosing condition of the skin that is being described increasingly in patients with renal diseases, many of whom are on dialysis therapy or have undergone renal transplantation. Its etiopathology is unknown, and no standard therapy currently exists. We describe a patient with NFD for whom histopathologic studies indicated that the fibrotic process affected subcutaneous tissue, striated muscles, diaphragm, pleura, pericardium, great vessels of the heart, left ventricle and septum, and tunica albuginea in addition to the dermis. Fibroblast-like cells positive for CD34 and CD45RO and scattered CD68-positive cells were found in affected tissues. The presentation of our case is unusual in that the disease process started in the lower abdomen and upper extremities and involved the upper extremities to a greater extent than the lower extremities. Our findings indicate that the fibrosis associated with NFD can extend beyond dermis and, as part of a systemic fibrosing disorder, can involve subcutaneous tissues, striated muscles, diaphragm, pleura, pericardium, and myocardium. We therefore suggest that "nephrogenic systemic fibrosis" would be a more appropriate term for this disease entity.

Original languageEnglish (US)
Pages (from-to)754-759
Number of pages6
JournalAmerican Journal of Kidney Diseases
Volume46
Issue number4
DOIs
StatePublished - Oct 1 2005

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Nephrogenic Fibrosing Dermopathy
Striated Muscle
Pleura
Pericardium
Subcutaneous Tissue
Dermis
Diaphragm
Upper Extremity
Heart Ventricles
Heart Septum
Abdomen
Kidney Transplantation
Dialysis
Lower Extremity
Myocardium
Fibrosis
Fibroblasts
Kidney
Skin
Therapeutics

Keywords

  • Dialysis
  • End-stage renal disease (ESRD)
  • Fibrosis
  • Nephrogenic fibrosing dermopathy (NFD)
  • Systemic fibrosis

ASJC Scopus subject areas

  • Nephrology

Cite this

Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis : Report of a new case with literature review. / Daram, Sumanth Reddy; Cortese, Cherise M.; Bastani, Bahar.

In: American Journal of Kidney Diseases, Vol. 46, No. 4, 01.10.2005, p. 754-759.

Research output: Contribution to journalArticle

Daram, Sumanth Reddy ; Cortese, Cherise M. ; Bastani, Bahar. / Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis : Report of a new case with literature review. In: American Journal of Kidney Diseases. 2005 ; Vol. 46, No. 4. pp. 754-759.
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