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Eosinophilic Fasciitis & Eosinophilia-Myalgia Syndrome

Disorders similar but not the same

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Updated December 16, 2003

Eosinophilic fasciitis: What is it?
Eosinophilic fasciitis (EF) is a disorder that causes inflammation and thickening of the skin and tissue under the skin called fascia (which covers the surfaces of muscles and other tissues). The inflammation is caused by a type of white blood cell (eosinophil) which is present in abnormally high numbers. The cause is not known, and it is rare in the United States. Diagnosis is made by looking at a sample (biopsy) of affected skin. It is thought that EF is a type of scleroderma.

What are the symptoms?
The fascia and skin swell and thicken, causing the following symptoms:

  • Swelling and puckering of the skin, starting in the arms and legs; may look bumpy like the skin of an orange
  • Aching of the arms and legs and arthritis in the hands and wrists
  • Restriction of movement of hands, wrists, elbows, ankles, and shoulders, sometimes to the point where they can't move at all

How is it treated?
The goal of treatment is to reduce or eliminate the inflammation, so aspirin or non-steroidal anti-inflammatory drugs (NSAIDs, such as ibuprofen or naprosyn) are used. In more severe cases, prednisone may be used, along with immune suppression medications such as cyclophosphamide (Cytoxan) and methotrexate.

Eosinophilia-myalgia syndrome: What is it?
Eosinophilia-myalgia syndrome (EMS) is a disorder that causes inflammation by eosinophils in nerve, muscle, and connective tissue, which may include the fascia (as in EF).

Poisoning discovered
EMS was first recognized in 1989, when three women in New Mexico (U.S.) sought medical treatment for sets of similar symptoms. These women had all taken a health supplement, L-tryptophan, of a particular brand which was discovered to be contaminated. The sale of all brands of L-tryptophan were banned right away, but not before people died and thousands were affected by EMS.

There have been cases of EMS reported that are not linked to taking L-tryptophan.

What are the symptoms?
The most difficult symptom of EMS is generalized, severe muscle pain that tends to worsen over weeks and may cause muscle spasms. Other symptoms include:

  • Swelling of the arms and legs, and sometimes the face
  • Joint pain
  • Skin rash, skin changes, and hair loss
  • Cough and shortness of breath
  • Fatigue
EMS is sometimes misdiagnosed as fibromyalgia, chronic fatigue syndrome, lupus erythematosus, or arthritis.

How is it treated?
There is no cure for EMS, so treatment focuses on relieving the symptoms with medicines such as muscle relaxants and pain relievers. Prednisone helps some people but not all. This is a chronic (long-term) illness; a study of 333 people with EMS showed after 4 years only 10% reported full recovery.

Information for this article was taken from:
- Sairam, S., & Lisse, J. Eosinophilia-myalgia syndrome. eMedicine Journal, Vol. 3 No. 1.
- Nasef, S., & Lohr, K. Eosinophilic fasciitis. eMedicine Journal, Vol. 3 No. 5.
- Shiel, W. C. Eosinophilic fasciitis (Shulman's syndrome). Available online at MedicineNet.com.

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