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 may also occur.

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

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 ; 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 , 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 Eosinophilia Eosinophilia is defined as a peripheral blood eosinophil count > 500/mcL (> 0.5 × 10⁹/L). Causes and associated disorders are myriad but often represent an allergic reaction or... read more (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 Monoclonal Gammopathy of Undetermined Significance (MGUS) Monoclonal gammopathy of undetermined significance (MGUS) is the production of M-protein by noncancerous plasma cells in the absence of other manifestations typical of multiple myeloma. (See... read more that rarely progresses to frank hematologic malignancy (1 Diagnosis reference 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.... read more ). 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.

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 Prevention Pneumocystis jirovecii is a common cause of pneumonia in immunosuppressed patients, especially in those infected with human immunodeficiency virus (HIV) and in those receiving systemic... read more ), and vaccines against common infections (eg, streptococcal pneumonia Overview of Pneumonia Pneumonia is acute inflammation of the lungs caused by infection. Initial diagnosis is usually based on chest x-ray and clinical findings. Causes, symptoms, treatment, preventive measures, and... read more , influenza Influenza Influenza is a viral respiratory infection causing fever, coryza, cough, headache, and malaise. Mortality is possible during seasonal epidemics, particularly among high-risk patients (eg, those... read more , COVID-19 COVID-19 Vaccine COVID-19 vaccines provide protection against COVID-19, the disease caused by infection with the SARS-CoV-2 virus. Vaccination is the most effective strategy to prevent severe illness and death... read more ).