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Eosinophilic fasciitis

INTRODUCTION

Eosinophilic fasciitis (EF, also called diffuse fasciitis with eosinophilia) is an uncommon disorder of unknown etiology and poorly understood pathogenesis [1]. EF is characterized in its early phase by limb or trunk erythema and edema and later by collagenous thickening of the subcutaneous fascia. Eosinophilia is a prominent laboratory finding in the early phase. This topic will review the clinical manifestations, pathology, diagnosis, differential diagnosis, and treatment of EF.

CLINICAL MANIFESTATIONS

Eosinophilic fasciitis (EF) is characterized by symmetrical induration of the skin [2,3]. The onset is typically acute, and findings include erythema, swelling, and induration of the extremities that is accompanied by eosinophilia [4]. A subacute course may also occur. The thickening and hide-bound quality of the affected skin is somewhat similar to that seen with the scleroderma-spectrum disorders.

Cutaneous — Nearly all patients with EF have clinically apparent symmetrical skin involvement. There is a temporal evolution of skin findings. Initially, there may be marked, non-pitting edema of the extremities. With progression of the disease, the swelling resolves and is replaced by symmetrical induration with puckering that gives the skin the texture of orange peel (peau d’orange). While the initial edematous phase may be indistinguishable from early sclerodermatous skin changes, the irregular, woody, peau d’orange texture of EF is distinct from the smooth, shiny skin surface seen in patients with systemic sclerosis or localized scleroderma (picture 1). Skin involvement most commonly occurs on the extremities, neck, and trunk. Sclerodactyly, the hallmark of systemic sclerosis, is absent in EF. Moreover, the skin of the hands and feet is generally spared.

Elevation of an affected limb, which reduces the distending venous pressure, causes visible indentation along the course of the superficial veins. This physical finding, referred to as the “groove sign,” is probably due to the relative sparing of the epidermis and superficial dermis by the fibrotic process coupled with the relative immobility of the connective tissue around the remainder of the veins. Thus, the superficial layers of skin can bow inward as the peripheral venous pressure falls.

Articular — Inflammatory arthritis occurs in a substantial minority of patients with EF. In one study of 52 patients, 40 percent had arthritis [5]. However, limited joint mobility may also be the result of thickening and loss of pliability of overlying skin and fascia. Joint contractures may result, sometimes occurring in the absence of typical skin involvement [6]. In the series of 52 patients cited earlier [5], 56 percent had flexion contractures of the joints.

                            

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Literature review current through: Jul 2014. | This topic last updated: May 29, 2014.
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