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Nephrogenic systemic fibrosis (NSF, originally called ‘nephrogenic fibrosing dermopathy’) is a painful and debilitating fibrosing disorder that was first identified in 1997 in several patients with stage 5 chronic kidney disease (glomerular filtration rate (GFR) <15 ml/min/1.73 m2 or permanently requiring dialysis) who had undergone renal transplantation at Sharp Memorial Hospital in San Diego, California.1 It typically manifests with skin tightening, tethering and hyperpigmentation on the trunk and on the extremities, progressing from distal to proximal, and often results in fixed flexion contractures of the fingers, elbows, knees and ankles. More recently, extensive systemic involvement has been identified including fibrosis of lymph nodes, thyroid, oesophagus, heart, lungs, liver, diaphragm, skeletal muscle, genitourinary tract and dura mater.2 3 The presence of skin changes of NSF has been associated with a nearly threefold increased risk of death within 24 months.4
On skin biopsy, the dermis is hypercellular with increased numbers of dermal fibroblasts.5 6 Thickened collagen bundles aligned with intact elastic fibres extend from the superficial dermis through the subcutis along interlobular septa.6 Interstitial mucin deposition is increased. CD34 fibrocytes, presumably originating from the circulation, and occasional cells of monocyte–macrophage lineage that stain for factor XIIIa and CD68 may be present in the dermis. When deeper tissue is sampled, fibrosis of subcutaneous tissue and fascia can also be observed.7 However, unlike scleroderma or scleromyxoedema, there is little or no inflammatory infiltrate in the skin of patients with NSF. Although the skin changes and joint contractures of NSF may resemble those of chronic graft-versus-host disease, NSF occurs in the absence of recent allogeneic haematopoietic cell transplantation; on skin biopsy, increased interstitial mucin and CD34 fibrocytes in the dermis typically differentiate NSF from chronic graft-versus-host disease.8
In 2006, Grobner reported that five of nine patients receiving …
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