Evaluate the effect of high dose MTX on skin induration in patients with eosinophilic fascia.

Eosinophilic fasciitis (EF)is a fibrosing skin disorder extensively involving the deep fascia. The aim of this pilot study is to evaluate the effect of high dose MTX on skin induration in patients with EF. In addition, we study the effect of this treatment on joint mobility, peripheral blood eosinophilia, functional ability, and pulmonary function. By administering high dose MTX intravenously every four weeks with a Leukovorin rescue, we hope to induce a more pronounced and sustained effect on the involved skin with less (long-lasting) side effects.

Inclusion Criteria:

• Eosinophilic fasciitis as defined by clinical judgement of an expert,

• Histologically confirmed with a skin-fascia-muscle biopsy, and in additon:

  • Either the presence of a modified skin score according to Zachariae of 8 or higher (0-18), regarded as a generalised eosinophilic fasciitis or the presence of severe contractures due to eosinophilic fasciitis lesions.

Exclusion Criteria:

• Age < 18 yrs
• Contraindications to MTX: AST/ALT level > 2 times upper limit of normal, WBC count < 3.5 x 109/l or platelet count < 150 x 109/l, serum creatinine > 130 micromol/l or clearance < 50 ml/min, confirmed by two repeated tests within one month.
• Chronic liver disease, insulin dependant diabetes mellitus, alcohol abuse
• Restrictive pulmonary disease (total lung capacity or vital capacity < 40% of predicted) or interstitial lung disease (KCO < 60% of predicted)
• Pregnancy or child bearing potential without adequate contraception
• The presence of any serious co-morbidity or malignancy
• Use of other anti-folate drugs than MTX