Eosinophilic Fasciitis Treatment & Management

  • Author: Peter M Henning, DO, MAJ, MC; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Jan 18, 2012
 

Medical Care

When considering medical therapies for eosinophilic fasciitis (EF), especially second-line agents, it should be noted that up to one third of eosinophilic fasciitis cases may spontaneously resolve.[52]

  • Initial therapy[17, 18, 19, 38, 48, 53]
    • There is wide consensus that systemic corticosteroids are the initial therapeutic agent of choice. Typically, prednisone or prednisolone is used, in doses ranging from 20-100 mg/day.
    • Response is considered satisfactory with reduction in edema, improvement in skin thickening, resolution of carpal tunnel syndrome, and gradual decrease in joint contracture.
    • Eosinophilia and inflammatory markers frequently resolve promptly after initiation of prednisone therapy.
  • Additional therapeutic agents and adjunctive therapies[18, 54, 55, 56, 57]
    • Multiple additional therapeutic agents have been used as disease-modifying or steroid-sparing agents in persistent or steroid-resistant cases of eosinophilic fasciitis. It should be noted that there is no general consensus with regard to the best agent for this type of disease. Treatment numbers are generally small, and controlled trials are lacking.
    • Case reports detail the use of multiple additional agents, including antihistamines, cimetidine, hydroxychloroquine, chloroquine, azathioprine, cyclosporine, dapsone, infliximab, tacrolimus, methotrexate, D-penicillamine, griseofulvin, ketotifen, and alpha-interferon, with varying rates of response. Some recent data suggest that other anti–tumor necrosis factor (TNF)–alpha agents may also be beneficial.[52]
    • Physical therapy should be initiated to improve joint mobility and to decrease contractures.
    • Surgical release has been used in some cases to manage significant joint contractures.[58]
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Surgical Care

Surgical release has also been used in some cases of eosinophilic fasciitis for significant joint contractures.[58]

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Consultations

Dermatologists, rheumatologists, and surgeons (for the skin-muscle biopsy) are consulted most often.

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Contributor Information and Disclosures
Author

Peter M Henning, DO, MAJ, MC  Fellow, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC

Peter M Henning, DO, MAJ, MC is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

George R Mount, MD, MAJ USA MC  Assistant Professor of Medicine, Uniformed Services University of the Health Sciences; Attending Physician, Department of Rheumatology, Madigan Army Medical Center, Tacoma, WA

George R Mount, MD, MAJ USA MC is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Nicholas David Kortan, DO, CPT, MC,  Resident Physician, Department of Internal Medicine, Walter Reed Army Medical Center, Washington, DC

Nicholas David Kortan, DO, CPT, MC, is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Sherif Nasef, MD  Consulting Staff, Department of Internal Medicine, Division of Rheumatology, Lake Havasu Regional Medical Center

Sherif Nasef, MD is a member of the following medical societies: American College of Physicians, American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, and American Medical Association

Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS  Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

John Varga, MD  Professor, Department of Internal Medicine, Division of Rheumatology, Northwestern University

John Varga, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Central Society for Clinical Research, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Additional Contributors

The opinions or assertions contained here are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.

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The arm of this patient demonstrates the puckered, so-called orange-peel or cobblestone skin that may occur in eosinophilic fasciitis.
The skin of the patient's back appears shiny due to the stretched dermis overlying an inflamed fascia. Mild diffuse hyperpigmentation is present, along with a U-shaped area of hypopigmentation extending approximately from T10 to L4.
The skin of the abdomen and breasts is shiny and taut. The thigh reveals puckering or cobblestoning of the overlying dermis due to scattered retraction from scarred fascia.
Eosinophilic fasciitis. Top: In this gross specimen, the dermis (A), subcutaneous adipose tissue (B), and skeletal muscle do not appear unusual. However, the fascia (D) is markedly thickened. Bottom left: The gross findings are recapitulated in this low-power photomicrograph. The epidermis, dermis (A), and subcutaneous adipose tissue are not remarkable in this case. The fascia (D) is markedly thickened and focally infiltrated by inflammatory cells (E). The small amount of skeletal muscle (C) appears normal (hematoxylin and eosin stain at low power). Bottom right: A close-up photograph of a portion of the fascia showing mostly edematous cellular connective tissue (F). It is focally infiltrated by inflammatory cells, including lymphocytes, plasma cells, and histiocytes. The more intensely stained hypocellular pink bands across the top of the field (G) are part of an interstitial exudate of fibrin (hematoxylin and eosin stain at medium power).
 
 
 
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