• Daniel Erez, Yehuda Shoenfeld, Ayman Natour, Zamir Dovrish, Oshrat E Tayer-Shifman, and Yair Levy.
• Department of Medicine D, Meir Medical Center, Kfar Saba.
• Medicine (Baltimore). 2021 Apr 2; 100 (13): e25359e25359.

RationaleEosinophilic fasciitis (EF) is an uncommon connective tissue disorder characterized by limb and trunk erythema, with symmetrical thickening of the skin. Its pathogenesis is poorly understood. Treatment consists mainly of glucocorticoids. Yet, no randomized trials have evaluated therapies for this rare disease and the optimal treatment modality remains unclear. Although most patients show partial or complete response to glucocorticoids, many relapse upon drug tapering, while others either do not respond at all or fail to sustain prolonged remission. Second-line therapy for this rare disorder includes mainly methotrexate (MTX), azathioprine, cyclosporine and hydroxychloroquine. Recently, several attempts using rituximab and intravenous immunoglobulins (IVIG) have shown good clinical results.Patient ConcernsThe three patients had good clinical response to glucocorticoid treatment, followed by disease flare when the drug dose was tapered. Adding methotrexate in all patients and azathioprine to patient 3 did not lead to remission.DiagnosesEF was diagnosed in all patients based on clinical presentation accompanied by fascia biopsy that demonstrated eosinophilic fasciitis.InterventionsThe patients were successfully treated with rituximab or IVIG, achieving sustained remission.OutcomesThe three cases had good clinical response to glucocorticoid treatment, followed by disease flare when the drug dose was tapered. The patients were then successfully treated with rituximab or IVIG, achieving sustained remission.LessonsThis review of three cases of EF supports the results of previous reports, suggesting addition of rituximab and IVIG is an effective treatment for patients with refractory disease.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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