Eosinophilic Fasciitis

Eosinophilic fasciitis (EF) is an uncommon scleroderma-like syndrome with an unknown etiology and pathogenesis. Painful swelling with progressive induration and thickening of the skin and soft tissues of the limbs and trunk are the clinical hallmarks of the disease. Peripheral blood eosinophilia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate are the main laboratory findings. Full-thickness wedge biopsy of the clinically involved skin is essential for establishing an accurate diagnosis. Differential diagnosis includes systemic sclerosis and other fasciitis syndromes caused by substances, such as L-tryptophan, myalgia-eosinophilia, and toxic oil syndrome. Diagnosis of EF can be established by clinical, laboratory, and histological findings. Tentative diagnostic criteria have been previously reported, although universally accepted criteria are lacking. Herein we update previously reported diagnostic criteria considering data published in the last decade. Corticosteroids are effective in the treatment of EF. Some patients improve spontaneously.