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.

What causes eosinophilic fasciitis?

Although the cause seems related to an inflammatory response, the agent(s) that trigger the response are not yet identified. In the 1980s, there was a toxic product in some lots of L-tryptophan, an over-the-counter sleep aide that was available at the time, which caused illness similar to eosinophilic fasciitis.

What are eosinophilic fasciitis symptoms and signs?

Eosinophilic fasciitis causes inflammation of the tissues beneath the skin as well as sometimes in the skin. This leads to symptoms of swelling, stiffness, warmth, and pain of the involved area. Occasionally, there is discoloration of the skin over the tissues affected and the skin can appear thicker than normal. Joint contractures occur in 50%-75% of patients.

The muscle of the involved area can become weakened. Muscle enzyme blood levels can be found to be elevated in the blood, particularly the enzymes aldolase and creatine phosphokinase (CPK).

How do health-care professionals diagnose eosinophilic fasciitis?

The diagnosis of eosinophilic fasciitis is made with a skin biopsy of a full thickness of involved deep skin tissue. The biopsy site is usually small, and the doctor numbs the area before the tissue is removed for study by a pathologist, dermatologist, or trained technician. In addition, the thickened fascia can be detected by MRI.

Medically Reviewed by a Doctor on 11/16/2015

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