The connective tissue is probably damaged by an autoimmune reaction.
Symptoms include pain, swelling, and inflammation of the skin.
A biopsy is done to remove tissue for examination and testing.
Corticosteroids are helpful.
The word eosinophilic refers to the initially high blood levels of a type of white blood cell called eosinophils White Blood Cells The main components of blood include Plasma Red blood cells White blood cells Platelets read more . The word fasciitis refers to inflammation of the fascia, which is the tough fibrous tissue that lies on top of and between the muscles.
The cause of eosinophilic fasciitis is unknown. The disorder occurs mainly in middle-aged men but may occur in women and children.
Symptoms of Eosinophilic Fasciitis
The usual initial symptoms of eosinophilic fasciitis are pain, swelling, and inflammation of the skin, particularly over the inside of the arms and the front of the legs. The skin of the face, chest, and abdomen may occasionally be affected. Typically, the fingers and toes are not affected.
Symptoms may first be noticed after strenuous physical activity. Symptoms usually progress gradually. After weeks, the inflamed skin begins to harden, eventually acquiring a texture similar to an orange peel.
As the skin gradually hardens, the arms and legs become difficult to move. Eventually, the arms and legs may become permanently frozen in unusual positions (contractures) if the disease is not treated early. Weight loss and fatigue are common. Muscle strength does not usually decrease, but muscle and joint pain may occur. Rarely, if the arms are involved, the person may develop carpal tunnel syndrome Carpal Tunnel Syndrome Carpal tunnel syndrome is a painful compression (pinching) of the median nerve as it passes through the carpal tunnel in the wrist. The cause of most cases of carpal tunnel syndrome is unknown... read more .
Sometimes, the numbers of red blood cells and platelets in the bloodstream become very low, resulting in fatigue and a tendency to bleed easily. Rarely, the person develops a blood disorder, such as failure to produce red blood cells (called aplastic anemia Aplastic Anemia Aplastic anemia is a disorder in which the cells of the bone marrow that develop into mature blood cells are damaged, leading to low numbers of red blood cells, white blood cells, and/or platelets... read more ), a low number of platelets (which help blood clot) that causes bleeding, or cancer of a specific type of white blood cells (lymphoma Overview of Lymphoma Lymphomas are cancers of lymphocytes, which reside in the lymphatic system and in blood-forming organs. Lymphomas are cancers of a specific type of white blood cells known as lymphocytes. These... read more ).
Diagnosis of Eosinophilic Fasciitis
A doctor suspects eosinophilic fasciitis because of its typical symptoms.
The diagnosis of eosinophilic fasciitis is confirmed by taking a biopsy of affected skin and fascia and testing the samples. The biopsy sample must include all skin layers down to the muscle.
Blood tests are also done. Blood tests show that the number of eosinophils in the blood and the erythrocyte sedimentation rate (ESR) are increased. (The ESR is a test for inflammation and measures the rate at which red blood cells settle to the bottom of a test tube containing blood.) These increases indicate inflammation. Although blood test results can help doctors diagnose eosinophilic fasciitis, they alone cannot confirm a definite diagnosis of eosinophilic fasciitis because sometimes the abnormalities they detect are present in healthy people or in people who have other disorders. The diagnosis of eosinophilic fasciitis is based on all of the information doctors gather, including symptoms, physical examination results, and all test results.
Magnetic resonance imaging Magnetic Resonance Imaging (MRI) In magnetic resonance imaging (MRI), a strong magnetic field and very high frequency radio waves are used to produce highly detailed images. MRI does not use x-rays and is usually very safe... read more (MRI) can also help make the diagnosis but is not as conclusive as biopsy.
Prognosis of Eosinophilic Fasciitis
The long-term outcome varies, but eosinophilic fasciitis may resolve after prompt treatment.
Treatment of Eosinophilic Fasciitis
Most people respond rapidly to high doses of the corticosteroid prednisone. Treatment of eosinophilic fasciitis should be started as early as possible to prevent scarring, tissue loss (atrophy), and contractures. Corticosteroids Corticosteroids: Uses and Side Effects do not cure tissue that is already atrophied and scarred. Doses are gradually reduced, but corticosteroids may need to be continued at low levels for 2 to 5 years. Some people need corticosteroids for long periods and possibly other drugs (for example, hydroxychloroquine or the immunosuppressants methotrexate, azathioprine, rituximab, mycophenolate mofetil, or cyclosporine). People may also be given nonsteroidal anti-inflammatory drugs Nonsteroidal Anti-Inflammatory Drugs Pain relievers (analgesics) are the main drugs used to treat pain. Doctors choose a pain reliever based on the type and duration of pain and on the drug's likely benefits and risks. Most pain... read more and histamine-2 blockers.
Contractures and carpal tunnel syndrome may need to be treated surgically.
Doctors continue to monitor people by doing blood tests so that, if a blood disorder develops, it can be diagnosed and treated as soon as possible.
People who are receiving immunosuppressants are also given drugs to prevent infections such as by the fungus Pneumocystis jirovecii (see prevention of pneumonia in immunocompromised people Prevention Pneumonia is infection of the lungs. Pneumonia in people whose immune system is weakened or impaired (for example, by acquired immunodeficiency syndrome [AIDS], cancer, organ transplantation... read more ).