Nephrogenic systemic fibrosis (nephrogenic fibrosis dermopathy, NSF/NFD)

Question 1

Pathogenesis of Nephrogenic systemic fibrosis:

Answer 1

• Fibrosis of skin and internal organs as a result of gadolinium-containing contrast exposure in the setting of acute kidney injury
• theory is that circulating fibrocytes (CD34 + Procollagen I) are recruited to skin leading to excess collagen and ECM production

Question 2

When does Nephrogenic systemic fibrosis typically occur after gadolinium-containing contrast exposure?

Answer 2

2-4 weeks after

Question 3

What are the skin findings of Nephrogenic systemic fibrosis?

Answer 3

symmetrically distributed painless, hyperpigmented and indurated “patterned plaques” (reticular or polygonal) on the extremities>trunk

• markedly “woody” induration with peau d’orange appearance
• linear banding due to focal areas of bound-down skin on proximal extremities
• scleral plaque (exam favorite): white-yellow plaques with dilated capillaries in patients <45 y/o

Question 4

What is classic eye finding in Nephrogenic systemic fibrosis?

Answer 4

white-yellowish scleral plaque

Question 5

Histopath of Nephrogenic systemic fibrosis?

Answer 5

similar to scleromyxedema, but fibrosis goes deeper into fat and fascia

• must obtain deep biopsy to fascia for dx
• increased CD34+ cells in dermis (morphea and scleroderma have decrease)

Question 6

Treatment of Nephrogenic systemic fibrosis?

Answer 6

Treat kidney disease

- often refractory to systemic steroids and immunosuppressives

• physical therapy to prevent contractures