Eosinophilic Fasciitis
(Shulman's Syndrome)
What are eosinophils?
Eosinophils are a particular type of white blood cells, usually representing a small percentage (less than 8% of the total white blood cell population). The number (eosinophil count) of these cells increases in certain illnesses, including allergies, asthma, Addison's disease, sarcoidosis, parasite infections, drug reactions, and connective tissue diseases (such as rheumatoid arthritis and scleroderma).
What is fascia?
The fascia is a lining tissue under the skin that covers a surface of underlying tissues. When the fascia is inflamed, the condition is referred to as "fasciitis."
What is eosinophilic fasciitis?
Eosinophilic fasciitis is a specific disease of the skin that leads to inflammation and thickening of the skin and fascia underneath. In patients with eosinophilic fasciitis, the involved fascia is inflamed with the eosinophil type of white blood cells. This leads to symptoms of progressive thickening, and often redness and warmth, and hardness of the skin surface.
Eosinophilic fasciitis is sometimes confused with the eosinophilia-myalgia syndrome and scleroderma.
How is eosinophilic fasciitis diagnosed?
The diagnosis of eosinophilic fasciitis is made with a biopsy of a full thickness of involved skin.
How is eosinophilic fasciitis treated?
Treatment of eosinophilic fasciitis is directed at eliminating the tissue inflammation and includes aspirin, other antiinflammatory drugs (NSAIDs), and cortisone. Many patients will improve spontaneously. Others can be afflicted with persistent tissue and joint pain, in addition to thickening of the involved tissues.
For aggressive eosinophilic fasciitis cortisone medications (such as prednisone and prednisolone) are considered along with immune suppression medications (such cyclophosphamide/Cytoxan and penicillimine/Depen, Cuprimine).
At the American College of Rheumatology meetings (November, 2001) the immune suppression drug, methotrexate, was demonstrated by research studies to provide an added benefit in treating eosinophilic fasciitis. This methotrexate research strongly suggests that methotrexate could be helpful in treating eosinophilic fasciitis. Only seven patients were reported, but all seven improved. Furthermore, their requirement for cortisone-related medications (steroids) was reduced.
Author: William C. Shiel Jr., MD, FACP, FACR
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