Eosinophilic fasciitis: a case series with an emphasis on therapy and induction of remission

Abstract

Eosinophilic fasciitis is an uncommon connective tissue disorder that affects patients of all ages, resulting in significant morbidity. Systemic corticosteroids can induce remission of disease. However, there is no universally accepted treatment ladder for eosinophilic fasciitis. This case series evaluates treatment efficacy in patients with eosinophilic fasciitis seen at Wake Forest University Department of Dermatology outpatient clinics. Patient charts were screened using ICD-9 diagnosis code 710.9 (unspecified diffuse connective tissue disease) to identify patients with eosinophilic fasciitis (n=10) seen at our institution. Patients were treated for an average 24 months with a combination of methotrexate and prednisone therapy, unless one or both were contraindicated, with each medication tapered conservatively to prevent disease flares. Alternate treatments included mycophenolate mofetil with prednisone, azathioprine with prednisone, prednisone monotherapy, and methotrexate monotherapy. Disease remission off therapy and on low-dose therapy was 66 and 70%, respectively. Our first-line therapy of concomitant methotrexate and prednisone is well-tolerated and effective for managing patients with eosinophilic fasciitis. Our study was limited to cases seen at a single academic institution.

Case of eosinophilic fasciitis during military training in a Nepalese British infantry soldier

“…It classically presents acutely with erythema and oedema of the extremities, progressing to induration of the skin and soft tissues, and, if untreated, patients can develop contractures. The classically described ‘groove sign’ of linear indentation around peripheral veins can be seen as induration worsens 4. The aetiology is unknown, although there is an association in the literature with strenuous exercise, with case series identifying this in a quarter to a half of cases 3 5.…”

“…The majority of early cases present with a peripheral blood eosinophila, often associated with a raised erythrocyte sedimentation rate(ESR) and hypergammaglobulinaemia 4. Diagnosis requires high clinical suspicion with characteristic changes on full thickness biopsy of fascia and muscle (thickened fascia with lymphocytic/eosinophilic infiltrates) 3.…”

“…Treatment consists of an extended, tapering course of systemic corticosteroids. The addition of methotrexate to this regimen appears to significantly reduce the risk of recurrence 4…”

Eosinophilic Fasciitis – Report of Three Cases and Review of the Literature

“…Other drugs that have been occasionally used are mycophenolate mofetil, cyclosporin A, dapsone, azathioprine, tumour necrosis factor-inhibitors, sirolimus, immunoglobulins, and D-penicillamine. Photo(chemo)therapy with either UVA1, PUVA or extracorporeal photochemotherapy has also been reported [12]. There are some case reports about the successful but off-label use of interleukin-6 antagonist tocilizumab [13], anti-CD-antibody rituximab [14], and Janus kinase inhibitor tofacitnib [15].…”

Refractory eosinophilic fasciitis successfully treated with infliximab: A case report

“…1,2 Systemic glucocorticoids are the first-line therapy. [1][2][3][4] Patients who fail to improve with glucocorticoids alone require an additional immunosuppressive agent. [1][2][3][4]…”