Saturday, January 01, 2011
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.
- 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.
- 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
- Hydroxyzine and cimetidine in standard doses can improve
swelling and pruritus.
- Hydroxychloroquine 200 mg twice daily may be useful alone or as
a steroid-sparing agent.
- PUVA, either with a topical or a systemic psoralen, has been
reported as effective.
- Photophoresis and other immunosuppressive regimens can be
considered for refractory and markedly symptomatic patients.
- 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.
- Inadequate (too superficial) biopsy may lead to the wrong
- Side effects of systemic steroids may be encountered and should
be minimized if possible.
- Cimetidine can produce signs of feminization (e.g.,
- In some patients, the fibrosis of the fascia does not
completely resolve, despite effective treatment.