Eosinophilic Fasciitis

Eosinophilic fasciitis is a rare and acute disease. It is characterized by symmetrical, painful inflammation, swelling and skin hardening. Eosinophilic fasciitis often occurs in middle adulthood.

Causes

To date, the causes for the occurrence of eosinophilic fasciitis have not been clarified. A connection with a defect of the immune system or a disproportionate physical strain or trauma is suspected. Also a connection with certain types of anaemia and thrombocytopenia, i.e. a lowering of the blood platelets, as well as Lyme disease is discussed. There are also great similarities with the “Eosinophiles-Myalgie-Syndrome”, which was first described in the 1980s and occurred after the intake of tryptophane.

Symptoms

In eosinophilic fasciitis, a severe inflammation develops in the tissue under the skin. This leads to eosinophilic fasciitis, which occurs symmetrically on the extremities, especially on the forearms. Rarely, the face or trunk are also affected. The muscles in the area affected by the fasciitis are weakened.

  • Swelling
  • Stiffness
  • Overheating
  • Pain
  • Redness and other discoloration
  • Thicker appearing skin

Diagnosis

A sample of all affected tissue layers can be used to determine whether eosinophilic fasciitis is present. For this purpose, the area is locally anesthetized and then the skin biopsy (sample) is taken and sent to the pathologist for detailed examination. In addition, the thickened fascia can often be detected by MRI imaging. In the laboratory, the muscle enzyme aldolase or the muscle enzyme creatine phosphokinase (CPK) may show abnormal values.

Therapy

The treatment goal in the therapy of eosinophilic fasciitis is to eliminate the inflammation in the tissue. Aspirin, other anti-inflammatory NSAIDs and cortisone are used for this purpose. In most patients, the symptoms improve relatively quickly with a high oral dose of cortisone.

A further, low-dose intake of cortisone preparations may be necessary for years to prevent a relapse. If aggressive eosinophilic fasciitis is present, it may be necessary to administer the cortisone preparation intravenously. In addition, immunosuppressive drugs (e.g. methotrexate, cyclophosphamide, penicillamine) may be given.