Eosinophilic fasciitis
Shulman syndromeEosinophilic 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.
Scleroderma
Scleroderma is a disease that involves the buildup of fibrous tissue in the skin and elsewhere in the body. It also damages the cells that line the ...
Read Article Now Book Mark ArticleCauses
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
Arthritis
Arthritis is inflammation or degeneration of one or more joints. A joint is the area where 2 bones meet. There are more than 100 different types of...
Read Article Now Book Mark Article - Carpal tunnel syndrome
Carpal tunnel syndrome
Carpal tunnel syndrome is a condition in which there is excessive pressure on the median nerve at the wrist. This is the nerve that allows feeling a...
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- 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)
ESR
ESR stands for erythrocyte sedimentation rate. It is commonly called a "sed rate. "It is a test that indirectly measures the level of certain protei...
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MRI
A magnetic resonance imaging (MRI) scan is an imaging test that uses powerful magnets and radio waves to create pictures of the body. It does not us...
Read Article Now Book Mark Article - Muscle biopsy
Muscle biopsy
A muscle biopsy is the removal of a small piece of muscle tissue for examination.
Read Article Now Book Mark Article - Skin biopsy (the biopsy needs to include the deep tissue of the fascia)
Skin biopsy
A skin lesion biopsy is when a small amount of skin is removed so it can be examined under a microscope. The skin is tested to look for skin conditi...
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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.
Leukemia
Leukemia is a type of blood cancer that begins in the bone marrow. Bone marrow is the soft tissue in the center of the bones, where blood cells are ...
Read Article Now Book Mark ArticleWhen 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.