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Eosinophilic fasciitis

Shulman syndrome

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.

Causes

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.

Symptoms

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

Exams and Tests

Tests that may be done include:

  • CBC with differential
  • Gamma globulins (a type of immune system protein)
  • Erythrocyte sedimentation rate (ESR)
  • MRI
  • Muscle biopsy
  • Skin biopsy (the biopsy needs to include the deep tissue of the fascia)

Treatment

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.

Outlook (Prognosis)

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

Possible Complications

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 to Contact a Medical Professional

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

Prevention

There is no known prevention.

References

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

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.

Lee LA, Werth VP. Skin and rheumatic diseases. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley and Firestein's Textbook of Rheumatology. 10th ed. Philadelphia, PA: Elsevier; 2017:chap 43.

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 www.ncbi.nlm.nih.gov/pubmed/24424187.

National Organization for Rare Disorders. Eosinophilic fasciitis. rarediseases.org/rare-diseases/eosinophilic-fasciitis/. Updated 2016. Accessed March 6, 2017.

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    • Superficial anterior muscles

      Superficial anterior muscles

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      • Superficial anterior muscles

        Superficial anterior muscles

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      Review Date: 1/10/2019

      Reviewed By: Gordon A. Starkebaum, MD, MACR, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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