Eosinophilic fasciitis is a rare disorder characterized by symmetric and rapidly progressive 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 but may be triggered by vigorous exercise or trauma, borreliosis, or exposure to certain medications and substances (1). Up to 10% of reported cases are associated with hematologic disorders such as aplastic anemia and multiple myeloma (2).
The disorder occurs mostly in middle-aged men but can occur in women and children.
References
1. Antic M, Lautenschlager S, Itin PH. Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature. Dermatology. 2006;213(2):93-101. doi:10.1159/000093847
2. Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. 1988;17(4):221-231. doi:10.1016/0049-0172(88)90008-x
Symptoms and Signs of Eosinophilic Fasciitis
The initial features are pain, swelling, and inflammation of the skin and subcutaneous tissues, followed by induration, creating a characteristic orange-peel configuration and vertical linear depression (groove sign) overlying superficial veins, most evident over the anterior surfaces of the extremities. The face and trunk are rarely 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. Distinct from systemic sclerosis, 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.
Internal organ involvement is uncommon (1).
Fatigue and weight loss are common.
Photo courtesy of Kinanah Yaseen, MD.
Symptoms and signs reference
1. Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. 1988;17(4):221-231. doi:10.1016/0049-0172(88)90008-x
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, Raynaud syndrome, abnormal nail-fold changes on capillaroscopy (including dilated capillary loops and dropouts as well as periungual hemorrhages), and visceral changes (eg, esophageal dysmotility). These manifestations are usually absent in eosinophilic fasciitis.
Diagnosis of eosinophilic fasciitis is confirmed by biopsy, which must 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, these patients seem to have low-risk monoclonal gammopathy that rarely progresses to frank hematologic cancer (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
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 a gradual reduction to 5 to 10 mg once a day as soon as the fasciitis resolves). Despite a scarcity of randomized 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 (1). If methotrexate is contraindicated, other medications such as azathioprine or mycophenolate mofetil can be used.
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).
Treatment reference
1. Mango RL, Bugdayli K, Crowson CS, et al. Baseline characteristics and long-term outcomes of eosinophilic fasciitis in 89 patients seen at a single center over 20 years. Int J Rheum Dis. 2020;23(2):233-239. doi:10.1111/1756-185X.13770
Prognosis for Eosinophilic Fasciitis
Although the long-term outcome varies, eosinophilic fasciitis is often self-limited after treatment.
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, 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.
