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.

Symptoms of Rheumatoid Arthritis

What are eosinophils?

Eosinophils are a particular type of white blood cells, usually representing a small percentage (less than 8% of the total white blood cell population) that are easily stained by eosin and other dyes; they have a characteristic double-lobed nucleus. The number of these cells (eosinophil count) increases in certain illnesses, including allergies, asthma, Addison's disease, sarcoidosis, parasite infections, drug reactions, and connective tissue diseases (such as rheumatoid arthritis and scleroderma). Eosinophilic fasciitis is sometimes referred to as Shulman's syndrome.

What is fascia?

The fascia is a sheet or band of fibrous connective tissue under the skin that covers a surface of underlying tissues. Fascia surrounds each of the muscles that move the skeleton. When the fascia is inflamed, the condition is referred to as "fasciitis."

What is eosinophilic fasciitis?

Eosinophilic fasciitis is a rare disease that leads to inflammation and thickening of the skin and fascia underneath. In patients with eosinophilic fasciitis, the involved fascia is inflamed with the eosinophil type of white blood cells. This leads to symptoms of progressive thickening and often redness, warmth, and hardness of the skin surface.

Occasionally, the onset of eosinophilic fasciitis follows a period of exertional physical activity. Eosinophilic fasciitis is sometimes confused with eosinophilia-myalgia syndrome and scleroderma. Eosinophilic fasciitis sometimes occurs associated with cancers such as leukemia and lymphoma.

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Eosinophilic Fasciitis Symptoms and Signs

Leg Swelling

Common causes of leg swelling include salt retention, cellulitis, congestive heart failure, and medication side effects. Less common causes of leg swelling include blood clots in the leg (thrombosis), parasite infection, lymphedema, liver disease, kidney disease, and diseases that cause thickness of the layers of skin, such as scleroderma and eosinophilic fasciitis. In these diseases, the leg swelling is typically characterized by nonpitting edema.

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.

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.

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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>.

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Reviewed on 11/16/2015
References
REFERENCE:

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

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