Eosinophilic fasciitis, also known as Shulman’s Syndrome) is an inflammatory disease that causes thickening of the skin as well as the fascia underneath the skin. Eosinophils are a type of white blood cells, and “eosinophilic” means a high number of white blood cells are present. Fascia is the tissue the overlays deeper tissue in the skin. Inflammation of the fascia is known as fasciitis. Eosinophilic fasciitis (EF) occurs when the inflamed fascia is infiltrated with white blood cells (eosinophils) producing tight, hard, red, and painful skin. It usually affects the arms and legs, but can also occur in the face, chest, and abdomen.

Cause of Eosinophilic Fasciitis

The exact cause of EF is not known. It seems to occur more in men between the ages of 40 and 50, but can affect women and children as well. EF is largely related to an autoimmune reaction that damages connective tissues in the body. Extraneous exercise can sometimes cause EF (Eosinophilic Fasciitis (Shulman's Syndrome), William C. Shiel Jr., MD, FACP, FACR, MedicineNet.com). It can also be associated with other medical conditions such as leukemia and lymphoma.

It is a slowly progressing disease that becomes worsened over a period of weeks. It graduates making it hard to move limbs. In some cases the limbs can contract in unusual positions with the patient unable to move them at all. EF can also bring about carpal tunnel syndrome in a number of patients (MedlinePlus, U.S. National Library of Medicine and National Institutes of Health). Many patients experience weight loss and fatigue, as well as joint and muscle pain.

Diagnosing Eosinophilic Fasciitis

The primary procedure for diagnosing EF is to perform a skin biopsy of an affected area. A blood test called erythrocyte sedimentation rate (ESR) is usually ordered to look for the presence of an abnormally high amount of inflammation in the body. Together with a complete history and physical, a doctor can generally make the diagnosis with assurance.

Treatment of Eosinophilic Fasciitis

Treatment of EF is aimed at controlling the inflammation of the fascia. Aspirin and nonsteroidal antiinflammatory drugs (NSAIDs) are used for this purpose. If the disease requires a more aggressive form of antiinflammatory effect, steroids may be ordered, such as prednisone. Some patients will respond to treatment modalities spontaneously, while others may not react to any treatment options. Methotrexate is a very potent drug that has been found beneficial in reducing the immune inflammation, and has just recently been discovered as a possible treatment for EF.

It is important to get regular checks at a doctor’s office, as he will want to monitor your white blood cell count and ESR on a regular basis. Reporting changes in symptoms is also important as sometimes treatment plans need to be altered.