Eosinophilic fasciitis

• Main Authors: Karolina Niklas, Arkadiusz Niklas, Mariusz Puszczewicz
• Format: Article
• Language: English
• Published: Index Copernicus International S.A. 2015-01-01
• Series: Postępy Higieny i Medycyny Doświadczalnej
• Subjects: eosinophilic fasciitis; Shulman disease; immunosuppressive treatment; eozynofilowe zapalenie powięzi; zespół Shulmana; leczenie immunosupresyjne
• Online Access: http://phmd.pl/gicid/01.3001.0009.6523

Eosinophilic fasciitis is a rare connective tissue disease with unclear etiology and pathogenesis. It is classified as a scleroderma-like syndrome. The disease is characterized by fibrosis of the skin and subcutaneous tissues with significant thickening of fascia. Visceral involvement is rare. Characteristic feature in laboratory tests is peripheral blood eosinophilia. Differential diagnosis should be performed, including ruling out systemic sclerosis, nephrogenic systemic fibrosis, eosinophilia-myalgia syndrome, scleromyxedema, hypereosinophilic syndrome or Churg-Strauss syndrome. Final diagnosis is confirmed by histopathological examination. In treatment of the disease corticosteroids and/or immunosuppressive drugs are used. Some other drugs showed activity in this disease e.g. dapsone, infiximab or rituximab. Prognosis is rather good but sometimes a long-term treatment is necessary. In this paper we summarized the current knowledge on eosinophilic fasciitis.