Еозинофільний фасциїт

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.

• 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 distal skin involvement often with telangiectasia, 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.

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 in about one third of patients. However, most of these patients have low risk monoclonal gammopathy that rarely progresses to frank hematologic malignancy (1). MRI, although not specific, can show thickened fascia, with the increased signal intensity in the superficial muscle fibers correlating with the inflammation.

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

• 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.

If combination immunosuppressive therapy is used, patients should be given prophylaxis for opportunistic infections, such as Pneumocystis jirovecii (see prevention of Pneumocystis jirovecii pneumonia), and vaccines against common infections (eg, streptococcal pneumonia, influenza, COVID-19).