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Eosinophilic Fasciitis

By

Alana M. Nevares

, MD, The University of Vermont Medical Center

Last full review/revision Feb 2020| Content last modified Feb 2020
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Eosinophilic fasciitis is an uncommon disorder characterized by symmetric and painful inflammation, swelling, and induration of the arms and legs. Diagnosis is by biopsy of skin and fascia. Treatment is with corticosteroids.

The cause of eosinophilic fasciitis is unknown. The disorder occurs mostly in middle-aged men but can occur in women and children.

Symptoms and Signs

The disease often begins after strenuous physical activity (eg, chopping wood). The initial features are pain, swelling, and inflammation of the skin and subcutaneous tissues, followed by induration, creating a characteristic orange-peel configuration most evident over the anterior surfaces of the extremities. The face and trunk are occasionally involved. Restriction of arm and leg movement usually develops insidiously. Contractures commonly evolve, secondary to induration and thickening of the fascia, but the process may also involve tendons, synovial membranes, and muscle. Typically, eosinophilic fasciitis does not involve the fingers and toes (acral areas). Muscle strength is unimpaired, but myalgia and arthritis may occur. Carpal tunnel syndrome may also occur.

Fatigue and weight loss are common. Rarely, aplastic anemia, thrombocytopenia, and lymphoproliferative processes develop.

Diagnosis

  • Biopsy

Eosinophilic fasciitis should be suspected in patients with typical symptoms. The cutaneous manifestations may suggest systemic sclerosis; however, patients with systemic sclerosis usually also have Raynaud syndrome and acral involvement, often with telangiectasia, and visceral changes (eg, esophageal dysmotility). These are usually absent in eosinophilic fasciitis.

Diagnosis of eosinophilic fasciitis is confirmed by biopsy, which should be deep enough to include fascia and adjacent muscle fibers. Characteristic findings are inflammation of the fascia, with or without eosinophils.

Blood tests are not diagnostic, but a complete blood count (CBC) usually shows eosinophilia (in early active disease), and serum protein electrophoresis shows polyclonal hypergammaglobulinemia. CBC should be done in all patients because the presence of eosinophilia helps in the diagnosis. Autoantibodies are usually absent. MRI, although not specific, can show thickened fascia, with the increased signal intensity in the superficial muscle fibers correlating with the inflammation.

Prognosis

Although the long-term outcome varies, eosinophilic fasciitis is often self-limited after treatment.

Treatment

  • Oral prednisone

Most patients respond rapidly to high doses of prednisone (40 to 60 mg once a day followed by gradual reduction to 5 to 10 mg a day as soon as the fasciitis resolves). Continued low doses may be required for 2 to 5 years. Some patients require longer courses and possibly other drugs (eg, hydroxychloroquine, methotrexate, azathioprine, rituximab, mycophenolate mofetil, cyclosporine). There are no controlled drug trials to guide therapy.

Nonsteroidal anti-inflammatory drugs (NSAIDs) and H2 blockers also have been used to treat eosinophilic fasciitis.

Surgical release of contractions and the carpal tunnel may be necessary.

Monitoring with CBCs is advised because hematologic complications occasionally occur. Prophylaxis for opportunistic infections, such as Pneumocystis jirovecii (see prevention of Pneumocystis jirovecii pneumonia), should be considered if combination immunosuppressive therapy is used.

Key Points

  • Patients develop symmetric and painful inflammation, swelling, and induration of the arms and legs with a characteristic orange-peel appearance.

  • Although cutaneous manifestations may suggest systemic sclerosis, patients with eosinophilic fasciitis usually do not have Raynaud phenomenon and acral involvement with telangiectasia, or visceral changes (eg, esophageal dysmotility).

  • Confirm the diagnosis with a biopsy that includes fascia and adjacent muscle.

  • Treat patients with prednisone, other immunosuppressants, or both.

Drugs Mentioned In This Article

Drug Name Select Trade
PLAQUENIL
CELLCEPT
OTREXUP
IMURAN
NEORAL, SANDIMMUNE
RAYOS
RITUXAN
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