What is eosinophilic fasciitis (EF)?
Eosinophilic fasciitis is a rare disease that leads to inflammation and thickening of the skin and fascia underneath. In patients with eosinophilic fasciitis, the involved fascia is inflamed with the eosinophil type of white blood cells. This leads to symptoms of progressive thickening and often redness, warmth, and hardness of the skin surface.
Occasionally, the onset of eosinophilic fasciitis follows a period of exertional physical activity. Eosinophilic fasciitis is sometimes confused with eosinophilia-myalgia syndrome and scleroderma. Eosinophilic fasciitis sometimes occurs associated with cancers such as leukemia and lymphoma.
What are eosinophils?
- Eosinophils are a particular type of white blood cells, usually representing a small percentage (less than 8% of the total white blood cell population) that are easily stained by eosin and other dyes; they have a characteristic double-lobed nucleus.
- The number of these cells (eosinophil count) increases in certain illnesses, including allergies, asthma, Addison's disease, sarcoidosis, parasite infections, drug reactions, and connective tissue diseases (such as rheumatoid arthritis and scleroderma).
- Eosinophilic fasciitis is sometimes referred to as Shulman's syndrome.
What is fascia?
- The fascia is a sheet or band of fibrous connective tissue under the skin that covers a surface of underlying tissues.
- Fascia surrounds each of the muscles that move the skeleton.
- When the fascia is inflamed, the condition is referred to as "fasciitis."
What causes eosinophilic fasciitis?
- Although the cause seems related to an inflammatory response, the agent(s) that trigger the response are not yet identified.
- In the 1980s, there was a toxic product in some lots of L-tryptophan, an over-the-counter sleep aide that was available at the time, which caused illness similar to eosinophilic fasciitis.
What are the symptoms of eosinophilic fasciitis?
- Eosinophilic fasciitis causes inflammation of the tissues beneath the skin as well as sometimes in the skin. This leads to symptoms of swelling, stiffness, warmth, and pain of the involved area. Occasionally, there is discoloration of the skin over the tissues affected and the skin can appear thicker than normal. Joint contractures occur in 50%-75% of patients.
- The muscle of the involved area can become weakened. Muscle enzyme blood levels can be found to be elevated in the blood, particularly the enzymes aldolase and creatine phosphokinase (CPK).
Diagnosis of eosinophilic fasciitis
- The diagnosis of eosinophilic fasciitis is made with a skin biopsy of a full thickness of involved deep skin tissue.
- The biopsy site is usually small, and the doctor numbs the area before the tissue is removed for study by a pathologist, dermatologist, or trained technician. In addition, the thickened fascia can be detected by MRI.
- Eosinophilic fasciitis is treated by pediatricians, internists, dermatologists, and rheumatologists; occasionally, surgeons are consulted for deep biopsies and joint contractures.
What is the treatment for eosinophilic fasciitis?
- Treatment of eosinophilic fasciitis is directed at eliminating the tissue inflammation and includes aspirin, other anti-inflammatory drugs (NSAIDs), and cortisone. Many patients will improve spontaneously. Others can be afflicted with persistent tissue and joint pain, in addition to thickening of the involved tissues.
- For aggressive eosinophilic fasciitis, cortisone medications (such as prednisone and prednisolone) are sometimes initially administered intravenously. Also considered are immune-suppression medications (such as methotrexate [Rheumatrex, Trexall], cyclophosphamide [Cytoxan], and penicillamine [Depen, Cuprimine]). More recently, mycophenolate mofetil (Cellcept) and rituximab (Rituxan) are being studied as potential therapies.
- Medical research has shown that immune-suppression drugs, such as methotrexate, can reduce both the immune inflammation and the need for continued cortisone medications.
What is the prognosis for eosinophilic fasciitis?
- The outlook for eosinophilic fasciitis is generally good, particularly if treated aggressively and early.
- Along with medications, physical therapy can be required for optimal rehabilitation.
- Poor function, however, is not uncommon, especially in children.
- Those with arthritis, atrophy of muscle, scarring limiting the joint range of motion (contracture), and shortening of limb length tend to do worse.
Is it possible to prevent eosinophilic fasciitis?
Because we do not yet know the cause of eosinophilic fasciitis, it cannot be prevented.
- CDC Warns of Potentially Fatal Bacterial Illness on U.S. Gulf Coast
- Helping Others as Volunteers Helps Kids 'Flourish': Study
- FDA Approves Pfizer's RSV Shot for Older Adults
- What to Do When Tough-to-Treat Lymphoma Strikes During Pregnancy
- Rate of Pregnant U.S. Women Who Have Diabetes Keeps Rising
- More Health News »
Health Solutions From Our Sponsors
Top Eosinophilic Fasciitis Related Articles
aspirinAspirin (Aspirin, Arthritis Foundation Safety Coated Aspirin, Bayer Aspirin, Bayer Children's Aspirin, Ecotrin, and many others) is a NSAID used to treat fever, pain, and inflammation in the body that results from forms of arthritis, and soft tissue injuries. Aspirin is also used for decreasing the risk of heart attacks and strokes. Side effects, drug interactions, pregnancy information, and pregnancy safety information should be reviewed prior to taking any medication.
Are Skin Rashes Contagious?Direct and indirect contact can spread some types of rashes from person to person. Rash treatment depends upon a rash's underlying cause. A rash that sheds large amounts of skin warrants urgent medical attention. Rashes can be either contagious or noncontagious. Noncontagious rashes include seborrheic dermatitis, atopic dermatitis, contact dermatitis, stasis dermatitis, psoriasis, nummular eczema, drug eruptions, hives, heat rash (miliaria), and diaper rash. Rashes usually considered contagious include molluscum contagiosum (viral), impetigo (bacterial), herpes (herpes simplex, types 1 and 2 viruses), rash caused by Neisseria meningitides (N. meningitides) (bacterial), rash and blisters that accompany shingles (herpes zoster virus), ringworm (fungal) infections (tinea), scabies (itch mite), chickenpox (viral), measles and rubella (viral), erythema infectiosum (viral), pityriasis rosea (viral), cellulitis and erysipelas (bacterial), lymphangitis (bacterial, and folliculitis (bacterial).
CancerCancer is a disease caused by an abnormal growth of cells, also called malignancy. It is a group of 100 different diseases, and is not contagious. Cancer can be treated through chemotherapy, a treatment of drugs that destroy cancer cells.
LeukemiaLeukemia is a type of cancer of the blood cells in which the growth and development of the blood cells are abnormal. Strictly speaking, leukemia should refer only to cancer of the white blood cells (the leukocytes) but in practice, it can apply to malignancy of any cellular element in the blood or bone marrow, as in red cell leukemia (erythroleukemia).
methotrexate (Rheumatrex, Trexall)
Methotrexate (Rheumatrex, Trexall) is a drug prescribed to treat cancer, psoriasis, inflammatory diseases of the skin, arthritis (rheumatoid arthritis in adults and children), psoriatic arthritis, polymyositis, lupus, and to induce miscarriage in women with ectopic pregnancies. Side effects drug interactions, dosage, and pregnancy safety information should be reviewed prior to taking this drug.
MRI (Magnetic Resonance Imaging Scan)MRI (or magnetic resonance imaging) scan is a radiology technique which uses magnetism, radio waves, and a computer to produce images of body structures. MRI scanning is painless and does not involve X-ray radiation. Patients with heart pacemakers, metal implants, or metal chips or clips in or around the eyes cannot be scanned with MRI because of the effect of the magnet.
What Is Non-Hodgkin's Lymphoma?Non-Hodgkin's lymphoma (NHL) is cancer of the lymphatic system, a vital part of the body's immune system. Symptoms include swollen lymph nodes, fever, night sweats, coughing, weakness, chest pain, unexplained weight loss, and abdominal pain.
Non-Itchy Red Spots: 20 Skin DisordersA variety of conditions can cause red spots, both itchy and non-itchy. Learn about common skin disorders, causes, and treatment.
Nonsteroidal Anti-inflammatory Drugs (NSAIDs)Nonsteroidal anti-inflammatory drugs (NSAIDs) are a class of drugs are used to treat inflammation, mild to moderate pain, and fever. Examples of the most common NSAIDs include: aspirin salsalate (Amigesic), diflunisal (Dolobid), ibuprofen (Motrin), ketoprofen (Orudis), nabumetone (Relafen), piroxicam (Feldene), naproxen (Aleve, Naprosyn,) diclofenac (Voltaren), indomethacin (Indocin), sulindac (Clinoril), tolmetin (Tolectin), etodolac (Lodine), ketorolac (Toradol), oxaprozin (Daypro), celecoxib (Celebrex).
penicillamine (Cuprimine, Depen)Penicillamine (Cuprimine, Depen) is an antirheumatic drug prescribed for the treatment of rheumatoid arthritis, Wilson's disease, lead poisoning, and The prevention of kidney stones in patients with cystinuria. Side effects, drug interactions, warnings and precautions, and patient information should be reviewed prior to taking any medication.
prednisolone (Orapred, Pediapred)Prednisolone (Flo-Pred, Pediapred, Orapred, Orapred ODT) is a corticosteroid prescribed to achieve prompt suppression of inflammation due to inflammatory and allergic conditions (for example, rheumatoid arthritis, lupus, gout, ulcerative colitis, Crohn's disease, hay fever, types of dermatitis, and many others. Side effects, drug interactions, dosage, storage, and pregnancy safety information should be reviewed prior to taking this medication.
Prednisone is a drug that belongs to the corticosteroid drug class, and is an anti-inflammatory and immune system suppressant. It's used to treat a variety of diseases and conditions, for example: inflammatory bowel disease (Crohn's disease and ulcerative colitis), lupus, asthma, cancers, and several types of arthritis.
Common side effects are weight gain, headache, fluid retention, and muscle weakness. Other effects and adverse events include glaucoma, cataracts, obesity, facial hair growth, moon face, and growth retardation in children. This medicine also causes psychiatric problems, for example: depression, insomnia, mood swings, personality changes, and psychotic behavior. Serious side effects include reactions to diabetes drugs, infections, and necrosis of the hips and joints.
SclerodermaScleroderma is an autoimmune disease of the connective tissue. It is characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body, leading to thickness and firmness of involved areas. Scleroderma is also referred to as systemic sclerosis, and the cause is unknown. Treatment of scleroderma is directed toward the individual features that are most troubling to the patient.
Skin BiopsyDuring a skin biopsy, a piece of skin is removed under a local anesthesia and examined using a microscope. The different types of skin biopsy include shave biopsy, punch biopsy, and excisional biopsy. Skin biopsies are performed to diagnose skin growths, skin conditions, and skin cancers.
Skin PictureThe skin is the largest organ of the body, with a total area of about 20 square feet. See a picture of the Skin and learn more about the health topic.
Skin Problems and Treatments: Causes of Skin LesionsStrange spots on your skin? Find out some of the common causes of skin lesions.
The Skin: 7 Most Important Layers and FunctionsThe skin is the largest organ in the body and it covers the body's entire external surface. It is made up of seven layers. The first five layers form the epidermis, which is the outermost, thick layer of the skin. The hypodermis is the deepest layer of skin situated below the dermis.