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Eosinophilic Fasciitis: an Updated Review on Diagnosis and Treatment

Abstract

Purpose of Review

First recognized in 1974, eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia with characteristic cutaneous and hematologic manifestations. This review discusses recent trends in the diagnosis and treatment of EF.

Recent Findings

Although fascial biopsy has classically been considered the gold standard for making a diagnosis of EF, radiologic imaging, particularly magnetic resonance imaging, has been increasingly used for both diagnosis and monitoring of treatment response. Systemic corticosteroids remain the first-line treatment for EF; however, their often prolonged use in the treatment of EF has prompted a search for adjunctive therapies. Methotrexate has emerged as the leading corticosteroid-sparing agent for EF.

Summary

Since EF was initially described over 40 years ago, important diagnostic and therapeutic progress has been made. Future efforts should be directed at the pursuit of prospective studies including clinical trials and evidence-based guidelines.

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Acknowledgements

Dr. Femia’s career has been in part supported by funding from the Noah-Sadie K. Wachtel Foundation. Dr. Vleugels’ career has been supported by a Medical Dermatology Career Development Award from the Dermatology Foundation.

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Correspondence to Ruth Ann Vleugels.

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This article is part of the Topical Collection on Orphan Diseases

Alisa N. Femia and Ruth Ann Vleugels are co-last authors.

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Mazori, D.R., Femia, A.N. & Vleugels, R.A. Eosinophilic Fasciitis: an Updated Review on Diagnosis and Treatment. Curr Rheumatol Rep 19, 74 (2017). https://doi.org/10.1007/s11926-017-0700-6

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Keywords

  • Eosinophilic fasciitis
  • Shulman disease
  • Groove sign
  • Corticosteroids
  • Methotrexate
  • Magnetic resonance imaging