Eosinophilic fasciitis (Shulman syndrome) Flashcards

1
Q

Population more commonly affected by eosinophilic fasciitis and age

A

Female and caucasian in fourth to sixth decade

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2
Q

What is the pathogenesis of eosinophilic fasciitis?

A
  • it is a rapid onset fibrosis of fascia
  • Unknown exact cause
  • but TGF-B levels markedly elevated
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3
Q

What are potential triggers of eosinophilic fasciitis?

A

a/w recent history of strenuous activity (30%)

  • trauma
  • borreliosis
  • statin use
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4
Q

How does eosinophilic fasciitis present?

A
  • rapid onset of symmetric edema/induration and pain in extremities a/w peripheral eosinophilia
  • spares the hands, feet and face (vs scleroderma)
  • skin progresses to Peau d’orange

- lacks raynauds (vs scleroderma)

  • can see dry river bed or groove sign= linear depressions of veins within indurated skin
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5
Q

Histopath of eosinophilic fasciitis:

A

Massive thickening and fibrosis of deep fascia (10 to 50x’s the normal width)

  • lymphoplasmacytic infiltrate +/- eos
  • eos occasionally present, but tissue eos not essential for dx (peripheral eos are the thing you’re looking for)
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6
Q

How do you diagnose eosinophilic fasciitis?

A
  • deep biopsy to fascia
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7
Q

What labs are useful to order in eosinophilic fasciitis?

A

-serum eos will show peripheral eosinophilia

  • SPEP will show hypergammaglobulinemia (20-70%)
  • MRI or CT can show fascial thickening, eliminating need for Bx
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8
Q

Treatment of eosinophilic fascitits:

A

Excellent response to systemic steroids (vs deep morphea)

  • can also use steroid sparing agents (HQC, cyclosporine, dapsone, MTX, TNF-alpha inhibitors)
  • physical therapy to prevent joint contractures
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9
Q

Prognosis and clinical course of eosinophilic fasciitis:

A
  • up to 1/3 may resolve spontaneously
  • good response to steroids seen after a few weeks
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