Eosinophilic fasciitis
Eosinophilic fasciitis is a rare inflammatory disorder of the deep fascia that mimics systemic sclerosis with symmetric woody induration and peau d'orange skin of the extremities, but classically spares the fingers and face and lacks Raynaud phenomenon, nailfold capillary changes, and scleroderma-specific autoantibodies. Peripheral eosinophilia and a full-thickness skin-to-fascia biopsy establish the diagnosis. First-line treatment is high-dose prednisone, with methotrexate added as a steroid-sparing agent for an inadequate response.
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What this article covers
- Overview
Definition: eosinophilic fasciitis is a rare fibrosing disorder of the deep fascia that produces scleroderma-like skin induration of the extremities while sparing the fingers and face, placing it…
- Epidemiology
Rare disease: eosinophilic fasciitis is consistently described as a rare condition; a validated incidence or prevalence figure is not established in the available reference material.
- Risk factors
Strenuous physical exertion: symptoms are characteristically worse with exercise; this is the only exacerbating association substantiated in the available reference material.
- Pathophysiology
Fascial target: the inflammatory infiltrate of lymphocytes, plasma cells, and eosinophils centers on the deep fascia rather than the dermis, which is why a full-thickness, skin-to-muscle biopsy, not…
- Clinical features
Symmetric extremity involvement: swelling followed by woody, peau d'orange induration of the proximal extremities (forearms, arms, and legs).
- Differential diagnosis
Diffuse cutaneous systemic sclerosis: distal and proximal skin thickening that extends to the face, chest, abdomen, and hands, commonly with internal organ fibrosis and interstitial lung disease…
- Diagnosis
No validated classification criteria exist; the diagnosis is established by biopsy on a background of the classic clinical picture: peau d'orange induration sparing the hands and face, peripheral…
- Management
First-line, high-dose glucocorticoids: prednisone 1 mg/kg/day PO once daily, up to a maximum of 80 mg/day; maintain the high dose for 4 to 6 weeks before beginning a taper guided by improvement in…
- Complications
Restricted joint range of motion: the tightly bound, woody induration of the skin and underlying soft tissue can limit movement at the wrists, elbows, ankles, and knees while active disease persists.
- Prognosis
Favorable with treatment: most patients respond within the 4- to 6-week glucocorticoid induction period, and induration and peripheral eosinophilia regress over the following months; the minority…
- Prevention
No primary prevention is defined: no established environmental, occupational, or hereditary exposure to modify is described in the available reference material beyond the association with strenuous exercise.
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