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Therapeutic Strategies

Eosinophilic Fasciitis

Timothy Berger Bruce Wintroub

Saturday, January 01, 2011

Overview

Eosinophilic fasciitis is distinguished from scleroderma by its acute onset; its lack of hand and foot or visceral involvement; the characteristic involvement of the extremities (usually distal legs and arms); its tendency to spontaneously remit after 2-5 years, and its exquisite responsiveness to systemic corticosteroid therapy. Patients often complain of weakness, pain, stiffness, and fatigue, and have swelling and sometimes limb contracture of the affected extremities. The pathogenesis of eosinophilic fasciitis is not known, and the therapeutic strategy is to suppress the inflammation that characterizes this disorder.

First Steps

  1. Prednisone 40-60 mg/day, given in a single morning dose, is usually dramatically effective. Extremity swelling and symptoms resolve quickly, whereas the fascial thickening may take months to improve and may never completely resolve. After improvement of the patients symptoms and swelling, slowly taper therapy over 6-18 months. Most patients can be completely weaned off steroids.
  2. The initial work-up consists of an incisional skin biopsy that extends to include the fascia; total eosinophil count; and determinations of the ESR. Hypergammaglobulinemia may also be found.

Alternative Steps

  1. Hydroxyzine and cimetidine in standard doses can improve swelling and pruritus.
  2. Hydroxychloroquine 200 mg twice daily may be useful alone or as a steroid-sparing agent.
  3. PUVA, either with a topical or a systemic psoralen, has been reported as effective.
  4. Photophoresis and other immunosuppressive regimens can be considered for refractory and markedly symptomatic patients.

Pitfalls

  1. Eosinophilic fasciitis must be distinguished from scleroderma. Recognition is critical because therapy and prognosis are radically different. Refractory cases of eosinophilic fascititis may represent morphea profunda.
  2. Inadequate (too superficial) biopsy may lead to the wrong diagnosis.
  3. Side effects of systemic steroids may be encountered and should be minimized if possible.
  4. Cimetidine can produce signs of feminization (e.g., gynecomastia).
  5. In some patients, the fibrosis of the fascia does not completely resolve, despite effective treatment.

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