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 Overview of Thrombocytopenia Thrombocytopenia is a low number of platelets (thrombocytes) in the blood, which increases the risk of bleeding. Thrombocytopenia occurs when the bone marrow makes too few platelets or when... read more (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 ).

The diagnosis of eosinophilic fasciitis is based on all of the information doctors gather, including symptoms, physical examination results, and all test results.

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.

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.

The long-term outcome varies, but eosinophilic fasciitis may resolve after prompt treatment.

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 a few years. Immunosuppressive drugs (such as methotrexate or, alternatively, azathioprine or mycophenolate mofetil) may be used in combination with corticosteroids.

Contractures and 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 may need to be treated surgically.

Physical therapy Physical Therapy (PT) Physical therapy, a component of rehabilitation, involves exercising and manipulating the body with an emphasis on the back, upper arms, and legs. It can improve joint and muscle function, helping... read more may help decrease and prevent more contractures.

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 ) and vaccines against common infections such as pneumonia Pneumococcal Vaccine Pneumococcal vaccines help protect against bacterial infections caused by Streptococcus pneumoniae (pneumococci). Pneumococcal infections include ear infections, sinusitis, pneumonia... read more , influenza Influenza Vaccine The influenza virus vaccine helps protect against influenza. Two types of influenza virus, type A and type B, regularly cause seasonal epidemics of influenza in the United States. There are... read more , and COVID-19 COVID-19 Vaccine Coronavirus disease 2019 (COVID-19) vaccines provide protection against COVID-19. COVID-19 is the disease caused by infection with the SARS-CoV-2 virus. There are multiple COVID-19 vaccines... read more .