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) and C-reactive protein (CRP)
- 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 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
Contact 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.
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 March 3, 2023.
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/.
Review Date: 1/25/2023
Reviewed By: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, NY and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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