Learn About Eosinophilic Fasciitis

What is the definition of Eosinophilic Fasciitis?

Eosinophilic fasciitis (EF) is a syndrome in which tissue under the skin and over the muscle, called fascia, becomes swollen, inflamed and thick. The skin on the arms, legs, neck, abdomen or feet can swell quickly. The condition is very rare.

EF may look similar to scleroderma, but is not related. Unlike scleroderma, in EF, the fingers are not involved.

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What are the alternative names for Eosinophilic Fasciitis?

Shulman syndrome

What are the causes of Eosinophilic Fasciitis?

The cause of EF is unknown. Rare cases have occurred after taking L-tryptophan supplements. In people with this condition, white blood cells, called eosinophils, build up in the muscles and tissues. Eosinophils are linked to allergic reactions. The syndrome is more common in people ages 30 to 60.

What are the symptoms of Eosinophilic Fasciitis?

Symptoms may include:

  • Tenderness and swelling of the skin on the arms, legs, or sometimes the joints (most often on both sides of the body)
  • Arthritis
  • Carpal tunnel syndrome
  • Muscle pain
  • Thickened skin that looks puckered
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What are the current treatments for Eosinophilic Fasciitis?

Corticosteroids and other immune-suppressing medicines are used to relieve symptoms. These medicines are more effective when started early in the disease. Nonsteroidal anti-inflammatory drugs (NSAIDs) may also help reduce symptoms.

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What is the outlook (prognosis) for Eosinophilic Fasciitis?

In most cases, the condition goes away within 1 to 3 years. However, symptoms may last longer or come back.

What are the possible complications of Eosinophilic Fasciitis?

Arthritis is a rare complication of EF. Some people may develop very serious blood disorders or blood-related cancers, such as aplastic anemia or leukemia. The outlook is much worse if blood diseases occur.

When should I contact a medical professional for Eosinophilic Fasciitis?

Call your health care provider if you have symptoms of this disorder.

How do I prevent Eosinophilic Fasciitis?

There is no known prevention.

Superficial anterior muscles
What are the latest Eosinophilic Fasciitis Clinical Trials?
Mepolizumab for the Treatment of Eosinophilic Fasciitis; An Open-Label, Single-Arm, Exploration Study

Summary: This is a proof of concept pilot study to investigate the efficacy of mepolizumab in the treatment of eosinophilic fasciitis.

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What are the Latest Advances for Eosinophilic Fasciitis?
Recalcitrant digital calcinosis cutis successfully treated with intralesional sodium thiosulfate under digital nerve blockade.
Response of recalcitrant generalized morphea to intravenous immunoglobulins (IVIg): three cases and a review of the literature.
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UVA-1 phototherapy as adjuvant treatment for eosinophilic fasciitis: in vitro and in vivo functional characterization.
Who are the sources who wrote this article ?

Published Date: January 31, 2021
Published By: Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Aronson JK. Tryptophan. In: Aronson JK, ed. Meyler's Side Effects of Drugs. 16th ed. Waltham, MA: Elsevier B.V.; 2016:220-221.

Florentino DF, Werth VP. Skin and rheumatic diseases. In: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 46.

James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM. Connective tissue diseases. In: James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 13th ed. Philadelphia, PA: Elsevier; 2020:chap 8.

National Organization for Rare Disorders. Eosinophilic fasciitis. rarediseases.org/rare-diseases/eosinophilic-fasciitis/. Updated 2016. Accessed May 10, 2021.

Pinal-Fernandez I, Selva-O' Callaghan A, Grau JM. Diagnosis and classification of eosinophilic fasciitis. Autoimmun Rev. 2014;13(4-5):379-382. PMID: 24424187 pubmed.ncbi.nlm.nih.gov/24424187/.