Nephrogenic Systemic Fibrosis: An Overview

Skin findings: brawny to erythematous papules and plaques with a “peau d’orange” texture.

Hand contractures in nephrogenic systemic fibrosis.

Ocular findings: white to yellow scleral plaques with variable vascular ectasia.

Histologic composite: (A) Scanning magnification showing dense collagen throughout the dermis (pink). (B) High magnification showing thick (dark pink) collagen bundles with surrounding clefts and lighter pink elastic fibers. Numerous spindle-cell nuclei are seen. (C) CD34 stain highlighting (brown) a dense network of spindle-cell processes. (D) Procollagen I stain among the same spindle-cell population. The dual positivity of CD34 and procollagen I identifies these cells as “circulating” fibrocytes.

Skeletal muscle involvement: normal skeletal fibers (left) and degenerating “beanbag”-like fibers (right).