Eosinophilic fasciitis (Shulman syndrome)

Question 1

Population more commonly affected by eosinophilic fasciitis and age

Answer 1

Female and caucasian in fourth to sixth decade

Question 2

What is the pathogenesis of eosinophilic fasciitis?

Answer 2

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

Question 3

What are potential triggers of eosinophilic fasciitis?

Answer 3

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

• trauma
• borreliosis
• statin use

Question 4

How does eosinophilic fasciitis present?

Answer 4

• 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

Question 5

Histopath of eosinophilic fasciitis:

Answer 5

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)

Question 6

How do you diagnose eosinophilic fasciitis?

Answer 6

• deep biopsy to fascia

Question 7

What labs are useful to order in eosinophilic fasciitis?

Answer 7

-serum eos will show peripheral eosinophilia

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

Question 8

Treatment of eosinophilic fascitits:

Answer 8

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

Question 9

Prognosis and clinical course of eosinophilic fasciitis:

Answer 9

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