Nephrogenic systemic fibrosis

Key Clinical Message

Nephrogenic systemic fibrosis (NSF) is a fibrosing disorder seen in patients with renal dysfunction, commonly precipitated by administration of gadolinium contrast. There is no consistently successful treatment, but oral steroids, topical dovonex, extracorporeal photopheresis, and plasmapheresis have been tried [http://www.icnfdr.org/ Last accessed: 1/6/2017]. Avoidance of gadolinium in such patients is the key to prevention.

Case Description

A 67‐year‐old male presented with painful nodular hardening of the skin over upper and lower limbs, gluteal region, and the back, 1 month after exposure to intravenous gadolinium contrast for magnetic resonance imaging during an episode of acute kidney injury, at which time his serum creatinine was ~4.2 mg/dL. This scan was done at a local hospital to rule out osteomyelitis of the foot in the setting of cellulitis, and apparently, the laboratory tests were not resulted prior to the scan. His serum gadolinium level at presentation was 13 ng/mL and biopsy of the right arm lesion was consistent with NSF with lesional tissue gadolinium level of 3.8 ng/mL. Despite hemodialysis and extracorporeal photopheresis, his skin disease progressed with painful nodules, calcified plaques in axillae, contractures affecting upper extremities (Fig. 1), and sacral region. He also developed oropharyngeal muscle involvement requiring feeding tube placement.

Figure 1.

(A) Left upper extremity showing skin thickening and calcified plaque in the axilla. (B) Right upper extremity showing skin thickening and erythematous nodules.

Informed Consent

Informed consent has been obtained for the publication of this clinical image.

Authorship

Both the authors made substantial contribution to the preparation of this manuscript and approved the final version for submission. AK: drafted the manuscript. VB: acquired the images and other pertinent patient data.

Conflict of Interest

The authors have declared that no conflict of interest exists.