A 62-year-old man with end-stage renal disease on haemodialysis develops progressive thickening and induration of the skin of his limbs and trunk, with yellow-orange pebbled plaques on the thighs and flexion contractures of the hips and knees within months. He had an MRI with contrast six months earlier. Skin biopsy shows thickened collagen bundles with increased dermal fibroblasts and elastic fibres without mucin. The most important step in management is:

• A High-dose oral corticosteroids
• B Plasmapheresis weekly
• C Avoidance of further gadolinium-based contrast exposure ✓
• D Penicillamine therapy

Explanation

Nephrogenic systemic fibrosis occurs almost exclusively in patients with severe renal impairment exposed to gadolinium-based contrast agents, particularly the less stable group II agents used historically. It causes fibrosing plaques with contractures and can involve internal organs. There is no reliably effective treatment, so prevention through avoidance of gadolinium in advanced renal failure, or use of the most stable macrocyclic agents with prompt dialysis afterwards, is the mainstay.

Reference: Rook's Textbook of Dermatology, 9th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.