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 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; 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.
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
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).
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.
