Eosinophilic fasciitis

What is eosinophilic fasciitis?

Eosinophilic fasciitis is a rare scleroderma-like disorder characterised by inflammation, swelling and thickening of the skin and fascia (fibrous tissue that separates different layers of tissues under the skin). It affects the forearms, the upper arms, the lower legs, the thighs, and the trunk (in order of decreasing frequency). The disease is considered by some to be a deep variant of the skin condition, morphoea, and is also known as pansclerotic morphoea with deep fascial involvement.

What causes eosinophilic fasciitis and who gets it?

The cause of eosinophilic fasciitis is unknown but it may have something to do with abnormal immune responses as hypergammaglobulinaemia and antinuclear antibodies are present. In addition, toxic, environmental, or drug exposures have been implicated.

It affects females and males, children and adults, with most cases occurring between the ages of 30 and 60 years.

What are the clinical features of eosinophilic fasciitis?

Patients usually present suddenly with painful, tender, swollen and red extremities. Within weeks to months patients develop stiffness and affected skin becomes indurated, creating a characteristic orange-peel appearance over the surfaces of the extremities. In severely affected areas, the skin and the subcutaneous tissue are bound tightly to the underlying muscle. This creates a woody-type appearance.

In 50% of cases, the disease is precipitated by an episode of strenuous physical exercise or activity. Other signs and symptoms that may be present include:

• Malaise, weakness, fever and weight loss
• Joint contractures of the elbows, wrists, ankles, knees and shoulders
• Carpal tunnel syndrome
• Inflammatory arthritis.

How is eosinophilic fasciitis diagnosed?

Laboratory studies in early active disease show eosinophilia, elevated ESR and polyclonal immunoglobulin (Ig)G. However, a full-thickness skin biopsy that includes the dermis, subcutaneous fat and fascia is necessary to confirm a diagnosis of eosinophilic fasciitis, which has specific pathological features.

What is the treatment for eosinophilic fasciitis?

Treatment of eosinophilic fasciitis is directed at preventing tissue inflammation. Oral corticosteroids are the mainstay of treatment, with most patients responding well to moderate-to-high doses of corticosteroids particularly if started early in the course of the disease. Continued low doses may be required for 2-5 years. In some cases, the disease may resolve spontaneously.

Other drugs that may be used in conjunction with corticosteroids include:

• Nonsteroidal anti-inflammatory agents (NSAIDs)
• Hydroxychloroquine
• Colchicine
• Cimetidine
• Azathioprine
• Ciclosporin
• Cyclophosphamide
• Methotrexate.

A physiotherapist is also important in the overall management of eosinophilic fasciitis to prevent and treat joint contractures.