Eosinophilic fasciitis (Shulman’s Syndrome)

Overview

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 of these cells (eosinophil count) 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).

Symptoms

Swelling of the skin * Morning stiffness * Paresthesias * Inflammatory arthritis * Claw like deformity of the hands

Causes

The cause of eosinophilic fasciitis is unknown. Persons with this condition have a build up of eosinophils, a type of white blood cell, into the affected fascia and muscles. Eosinophils are associated with allergic-type reactions, but their specific function is largely unknown. The syndrome is most common in people between ages 30 and 60. In some cases, it appears to be triggered by strenuous physical activity.

Prevention

There is no known prevention.

Prognosis

In most cases, the condition goes away within 3 to 5 years. However, symptoms may return (recur) or persist.

Treatment

Treatment with corticosteroid medications provides relief of the symptoms, especially when they are started early in the disease. Nonsteroidal anti-inflammatory drugs (NSAIDs) may also help relief symptoms.