Eosinophilic fasciitis (Shulman syndrome) Flashcards
Population more commonly affected by eosinophilic fasciitis and age
Female and caucasian in fourth to sixth decade
What is the pathogenesis of eosinophilic fasciitis?
- it is a rapid onset fibrosis of fascia
- Unknown exact cause
- but TGF-B levels markedly elevated
What are potential triggers of eosinophilic fasciitis?
a/w recent history of strenuous activity (30%)
- trauma
- borreliosis
- statin use
How does eosinophilic fasciitis present?
- 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
Histopath of eosinophilic fasciitis:
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)
How do you diagnose eosinophilic fasciitis?
- deep biopsy to fascia
What labs are useful to order in eosinophilic fasciitis?
-serum eos will show peripheral eosinophilia
- SPEP will show hypergammaglobulinemia (20-70%)
- MRI or CT can show fascial thickening, eliminating need for Bx
Treatment of eosinophilic fascitits:
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
Prognosis and clinical course of eosinophilic fasciitis:
- up to 1/3 may resolve spontaneously
- good response to steroids seen after a few weeks