66 - Eosinophilic fasciitis

Abstract

Eosinophilic fasciitis (EF) is a rare fibrosing disorder characterized by the rapid onset of symmetric induration of the extremities. Clinically, EF is marked by an early, edematous phase with erythema, followed by progressive sclerosis and a peau d’orange appearance. Ultimately, woody induration and stiffness of the limbs develop, and subsequent joint contractures are a major cause of morbidity. Laboratory investigations may reveal peripheral blood eosinophilia and an elevated erythrocyte sedimentation rate, but these are not essential in securing a diagnosis. The constellation of clinical, laboratory, imaging, and histologic findings establish the diagnosis of EF. Histopathologic examination from a full-thickness skin-to-muscle biopsy has been considered the gold standard for diagnosis. Magnetic resonance imaging is increasingly used by experts as a non-invasive modality to both establish the diagnosis and monitor the effectiveness of treatment. Additionally, EF has been reported in association with hematologic disorders; as such, abnormalities in complete blood count and immunofixation and/or protein electrophoresis may be seen. The primary goal of EF management is to halt disease progression. Softening of recently involved areas of sclerosis is possible with appropriate early treatment. Complete resolution of induration is rare and not routinely expected, particularly in patients with longstanding disease. Although responsive to oral steroids, EF will typically flare upon taper of steroids, and thus requires long-term treatment with a steroid-sparing immunosuppressive agent for durable clinical response and to avoid sequalae of prolonged steroid use. The combination of systemic corticosteroids and either methotrexate or mycophenolate mofetil should be considered first-line for EF. Additionally, physical therapy should be routinely recommended to prevent and/or improve joint contractures and related functional morbidity.