A 62-year-old woman with CKD-5D on hemodialysis for 5 years develops severe pruritus and patchy indurated skin plaques on the extremities and trunk. Skin biopsy shows dermal fibrosis with spindle cells positive for CD34. Which condition does she have, and what is the main precipitating factor?
- A Calciphylaxis; elevated calcium-phosphate product
- B Nephrogenic systemic fibrosis (NSF); gadolinium-based contrast agent exposure ✓
- C Lichen amyloidosis; amyloid deposition in dialysis patients
- D Scleroderma; anti-Scl-70 antibody
Explanation
Nephrogenic systemic fibrosis (NSF) occurs almost exclusively in patients with severe renal failure (eGFR <30 mL/min or ESKD) and is triggered by exposure to gadolinium-based contrast agents (GBCAs), particularly older linear GBCAs (gadodiamide, gadopentate). Gadolinium is retained in tissues and triggers TGF-β-mediated fibrosis. Skin biopsy shows dermal fibrosis with CD34-positive spindle cells (fibrocytes). Since the recognition of this association, macrocyclic GBCAs are preferred in CKD, drastically reducing NSF incidence. Calciphylaxis presents with painful skin necrosis/ulcers, not indurated plaques.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.