Eosinophilic Fasciitis (Shulman's Syndrome)

  • Medical Author:
    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

  • Medical Editor: Charles Patrick Davis, MD, PhD
    Charles Patrick Davis, MD, PhD

    Charles Patrick Davis, MD, PhD

    Dr. Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications.

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What is the treatment for eosinophilic fasciitis?

Treatment of eosinophilic fasciitis is directed at eliminating the tissue inflammation and includes aspirin, other anti-inflammatory drugs (NSAIDs), and cortisone. Many patients will improve spontaneously. Others can be afflicted with persistent tissue and joint pain, in addition to thickening of the involved tissues.

For aggressive eosinophilic fasciitis, cortisone medications (such as prednisone and prednisolone) are sometimes initially administered intravenously. Also considered are immune-suppression medications (such as methotrexate [Rheumatrex, Trexall], cyclophosphamide [Cytoxan], and penicillamine [Depen, Cuprimine]). More recently, mycophenolate mofetil (Cellcept) and rituximab (Rituxan) are being studied as potential therapies.

Medical research has shown that immune-suppression drugs, such as methotrexate, can reduce both the immune inflammation and the need for continued cortisone medications.

What specialists treat eosinophilic fasciitis?

Eosinophilic fasciitis is treated by pediatricians, internists, dermatologists, and rheumatologists; occasionally, surgeons are consulted for deep biopsies and joint contractures.

What is the prognosis (outlook) for eosinophilic fasciitis?

The outlook for eosinophilic fasciitis is generally good, particularly if treated aggressively and early. Along with medications, physical therapy can be required for optimal rehabilitation. Poor function, however, is not uncommon, especially in children. Those with arthritis, atrophy of muscle, scarring limiting joint range of motion (contracture), and shortening of limb length tend to do worse.

Is it possible to prevent eosinophilic fasciitis?

Because we do not yet know the cause of eosinophilic fasciitis, it cannot be prevented.

REFERENCE:

Henning, Peter M. "Eosinophilic Fasciitis." Medscape.com. Mar. 3, 2015. <http://emedicine.medscape.com/article/329515-overview>.

Medically Reviewed by a Doctor on 11/16/2015

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