[Eosinophllic fasciitis (Shulman disease)]

Abstract

Eosinophilic fasciitis is a rare connective tissue disease, described by Shulman in 1974. This syndrome is characterized by a symmetrical swelling of the skin associated with eosinophilia. A progressive induration of the skin replaces the swelling. Arms and legs are the most affected sites. The face and hands are usually not involved and the patients don't complain of Raynaud phenomenon. No circulating autoantibodies are found. Diagnosis is made with history, MRI and histology. MRI detects fascial thickening and contrast enhancement of this fascia. A full thickness skin to muscle biopsy is necessary to confirm the diagnosis. It shows an inflammation and thickening of the fascia with lymphocytes and plasmocytes. High doses of corticosteroids are reported to be effective in more than 70% of the cases. Immunosuppressive drugs are sometimes necessary to induce clinical remission.