Merck Manual

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Eosinophilic Fasciitis

By

Alana M. Nevares

, MD, The University of Vermont Medical Center

Reviewed/Revised Oct 2022
View PATIENT EDUCATION

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 of Eosinophilic Fasciitis

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 Carpal Tunnel Syndrome Carpal tunnel syndrome is compression of the median nerve as it passes through the carpal tunnel in the wrist. Symptoms include pain and paresthesias in the median nerve distribution. Diagnosis... read more Carpal Tunnel Syndrome may also occur.

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

Diagnosis of Eosinophilic Fasciitis

  • Biopsy

Eosinophilic fasciitis should be suspected in patients with typical symptoms. The cutaneous manifestations may suggest systemic sclerosis Diagnosis Systemic sclerosis is a rare chronic disease of unknown cause characterized by diffuse fibrosis and vascular abnormalities in the skin, joints, and internal organs (especially the esophagus... read more Diagnosis ; however, patients with systemic sclerosis usually also have distal skin involvement often with telangiectasia, Raynaud syndrome Raynaud Syndrome Raynaud syndrome is vasospasm of parts of the hand in response to cold or emotional stress, causing reversible discomfort and color changes (pallor, cyanosis, erythema, or a combination) in... read more Raynaud Syndrome , abnormal nail-fold changes on capillaroscopy (including ectatic capillary loops and dropouts as well as periungual hemorrhages), 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.

Diagnosis reference

  • 1. Mazori DR, Kassamali B, Min MS, et al: Characteristics and outcomes of eosinophilic fasciitis-associated monoclonal gammopathy. JAMA Dermatol 157(12):1508-1509, 2021. doi:10.1001/jamadermatol.2021.4302

Prognosis for Eosinophilic Fasciitis

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

Treatment of Eosinophilic Fasciitis

  • Oral prednisone

  • Methotrexate

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 a few years. Despite a scarcity of controlled drug trials to guide therapy, observational data have shown that the combination of corticosteroids and methotrexate is more likely to lead to complete treatment response than corticosteroids alone. If methotrexate is contraindicated, other drugs such as azathioprine or mycophenolate mofetil can be used as well.

Physical therapy may help with flexion contractures.

Monitoring with complete blood counts is advised because hematologic complications occasionally occur.

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

Key Points

  • Patients develop symmetric and painful 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 syndrome, distal skin involvement often with telangiectasia, nail-fold changes, or visceral changes (eg, esophageal dysmotility).

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

  • Treat patients with prednisone plus methotrexate or another immunosuppressant if methotrexate cannot be used.

Drugs Mentioned In This Article

Drug Name Select Trade
Deltasone, Predone, RAYOS, Sterapred, Sterapred DS
Jylamvo, Otrexup, Rasuvo, RediTrex, Rheumatrex, Trexall, Xatmep
Azasan, Imuran
CellCept, Myfortic
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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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