A 60-Year-Old Woman with Skin Thickening, Joint Contractures, and Peripheral Blood Eosinophilia

Abstract

Eosinophilic fasciitis (EF), also termed diffuse fasciitis with eosinophilia (DFE), is a disorder in the spectrum of skin fibrosing diseases characterized by woody induration of the skin involving the extremities, classically sparing the hands and feet. Patients with EF typically lack the visceral and autoimmune features characteristic of systemic sclerosis (SSc); specifically, EF patients do not typically manifest Raynaud’s phenomenon, gastroesophageal reflux, parenchymal lung or kidney disease, serologic abnormalities, for example, ANA or anti-topoisomerase I antibody, or abnormal nailfold capillary morphology. The skin involvement in EF can cause discomfort as well as be associated with joint contractures. Unlike SSc, EF patients generally respond well to glucocorticoid treatment. High-dose glucocorticoid therapy can provide substantial improvement or even resolution of the skin induration and thickening. Physical therapy is another key component in the management of patients with EF in order to avoid reduced function associated with joint flexion contractures. Immunosuppressive medications have also been used to treat patients with EF with mixed results.