A unique case of nephrogenic systemic fibrosis from gadolinium exposure in a patient with normal eGFR

Abstract

A 57-year-old woman presented with swelling and thickening of the skin of the lower extremities. Three months prior to presentation, patient had MRI with gadolinium as part of an evaluation for suspected pancreatic malignancy. Creatinine levels at the time of gadolinium exposure were 0.9-1.2 mg/dL, with a corresponding estimated glomerular filtration rate of 64 mL/min/1.73m² by modification of diet in renal disease equation. Twenty-four-hour urine creatinine clearance was performed as an outpatient following development of symptoms. This revealed a creatinine clearance of 23 mL/min, suggestive of advanced chronic kidney disease despite an estimated glomerular filtration rate of 64 mL/min/1.73m² Skin biopsy was positive for sclerosing dermopathy. These findings, in addition to the temporal association with gadolinium exposure, led to the diagnosis of nephrogenic systemic fibrosis.

Keywords: contraindications and precautions; dermatology; renal system.

Figure 1

Photograph of distal lower extremities revealing bilateral, hyperpigmented lesions with marked induration and linear banding. Alopecia and mild sclerodactyly are present. Coarse, fibrotic changes obscure distinct borders of medial malleolus.

Figure 2

(A) Low power view demonstrating fibrosis of the dermis with haphazardly arranged collagen bundles in the deep dermis. (B) High power view showing increased number of spindle-shaped fibroblasts with increased surrounding mucin deposition.

Figure 3

(A) Focal positivity for factor XIII and CD34 in the spindle cell proliferation present in the deep dermis. (B) Focal positivity for CD34 in the spindle cell proliferation present in the deep dermis.