Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature.

BACKGROUND: Eosinophilic fasciitis is a rare scleroderma-like illness. The clinical spectrum of the disease has evolved since its initial description.

METHODS: We identified all patients diagnosed with eosinophilic fasciitis over the past 10 years at our scleroderma clinic. Demographics, disease pattern, serologies, tissue pathology and reponse to treatment were all recorded.

RESULTS: Twelve patients with eosinophilic fasciitis were identified in our clinic over the past 10 years. The mean age at diagnosis was 49.8 +/- 9.8 years, with nine female and three male patients. The first symptoms were noticed at an average of 8.8 +/- 6.1 months before diagnosis. The mean initial absolute peripheral blood eosinophil count was 1188 +/- 1059 cells/L. Two patients had a monoclonal gammopathy, and two had positive ANA titers. All patients received corticosteroids, 10 of whom received the equivalent dose of > 20 mg/day of prednisone for more than a month. Five patients received hydroxychloroquine, two received methotrexate, one received cyclosporine, one received topical tacrolimus, and one received sulfasalazine. At a mean follow up of 17.6 months (range 2-94 months), 8 patients had a good response to treatment, 2 patients had no effect, and 2 patients had a poor response to treatment.

CONCLUSION: High dose corticosteroid treatment lasting longer than a month with or without an immunosuppressive agent helped most patients with eosinophilic fasciitis, best results seen in those patients who were initiated treatment early on after their first symptoms.