eosinophilic fasciitis (Shulman's syndrome)

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Introduction

A scleroderma spectrum disorder[2]

Etiology

Epidemiology

  • affects men & women equal
  • most commonly presents in 4th & 5th decades of life

Pathology

Clinical manifestations

Laboratory

Complications

Differential diagnosis

Management

  • prednisone 20-40 mg QD
    • most effective treatment
    • variable duration & efficacy of response
  • methotrexate
  • dermatology referral may be needed for biopsy & definitive diagnosis
  • surgery make be necessary to reduce contractures
  • prognosis:
    • spontaneous regression after 2-5 years may occur

More general terms

Additional terms

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 177, 876
  2. 2.0 2.1 2.2 2.3 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16 17, 18. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018.
  3. Ferri's Clinical Advisor, Instant Diagnosis and Treatment, Ferri FF (ed), Mosby, Philadelphia, 2003, pg 305
  4. Boin F, Hummers LK. Scleroderma-like fibrosing disorders. Rheum Dis Clin North Am. 2008 Feb;34(1):199-220 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18329541

Patient information

eosinophilic fasciitis patient information

Database