Eosinophilic Fasciitis (Ef)

When eosinophilic fasciitis (EF) is a rare disease, which is characterized by symmetrical, painful inflammation, swelling and induration of the arms and legs. The diagnosis is confirmed by biopsy of skin and muscle fascia. v therapeutic. a. Corticosteroids used.

The cause of the EF is unknown. The disease occurs v. a. in middle-aged men in front, but also women and children can relate to.

When eosinophilic fasciitis (EF) is a rare disease, which is characterized by symmetrical, painful inflammation, swelling and induration of the arms and legs. The diagnosis is confirmed by biopsy of skin and muscle fascia. v therapeutic. a. Corticosteroids used. The cause of the EF is unknown. The disease occurs v. a. in middle-aged men in front, but also women and children can relate to. Symptoms and signs The disease makes itself often after physically demanding activities (eg., Wood cases) noticeable. Pain, swelling and inflammation of the skin and subcutaneous tissue are the initial findings, followed by induration of the skin and the characteristic orange peel-like structure v. a. it must be held on the extensor surfaces of the extremities. Face and trunk are occasionally also affected. It comes to the gradual restriction of movement of arms and legs. After induration and Faszienverdickung often leads to contractures, and tendons, synovial membranes and muscles may be involved. Fingers and toes are typically spared. The muscular strength is unimpaired, but myalgia and arthritis can also occur as carpal tunnel syndrome. Fatigue and weight loss are common side findings, rarely leads to aplastic anemia, thrombocytopenia or lymphoproliferative diseases. Diagnostic biopsy The most important diagnostic indications are the typical symptoms of the disease. The skin manifestations can also think of a systemic sclerosis, but it also takes in patients here a Raynaud’s symptoms, acral involvement, telangiectasia and organ involvement (eg. As Ösophagusmotilitätsstörung). All this is absent in EF. The diagnosis is made by biopsy, which should be deep enough to capture skin, fascia and adjacent muscle fibers. Here, characteristically, a fasciitis with or without eosinophilia shows. The laboratory tests are non-specific, but can be found in early active disease eosinophilia in the blood and in the electrophoresis, a polyclonal gammopathy. A blood count should be performed in all patients, since the presence of eosinophilia helps in diagnosis. Autoantibodies are undetectable by MRI can be as nonspecific findings represent a thickened fascia and an inflammation-induced signal amplification in the superficial muscle fibers. The forecast long-term outcome appears variable, but usually the EF is self-limiting after treatment. Therapy oral prednisone Most patients respond promptly to a high dose of prednisone to (z. B. 40-60 mg / day po as an initial dose and subsequent reduction by 5-10 mg / day as soon as the fasciitis is declining). A subsequent continuous low-dose corticosteroid therapy may be necessary after 2-5 years. Some patients may require a longer course of treatment and possibly other drugs (eg. As hydroxychloroquine, methotrexate, azathioprine, rituximab, mycophenolate mofetil, cyclosporine). There are no controlled drug trials to guide therapy. NSAIDs and H2 blockers (eg., Cimetidine) also are used in the treatment of the EF used. A surgical exposure of contractures and the carpal tunnel may be necessary. Blood counts should be monitored because of the sometimes detectable hematologic complications. Prophylaxis against opportunistic infections such. Pneumocystis jirovecii should be done in addition, if a combined immunosuppressive therapy is used. Summary Patients develop a symmetric and painful inflammation, swelling and induration of the arms and legs with a characteristic orange peel-like structure. Although the skin manifestations can also think of a systemic sclerosis, can be found in patients with EF usually no Raynaud’s symptoms, acral involvement, telangiectasia and organ involvement (eg. As Ösophagusmotilitätsstörung). The diagnosis is confirmed by biopsy, including the fascia and adjacent muscles. Treatment is with prednisone and / or other immunosuppressants.

Health Life Media Team

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