Fasciite à éosinophiles (maladie de Shulman) : mise au point diagnostique et thérapeutique

摘要: Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by symmetrical and painful swelling with progressive induration thickening of the skin soft tissues. The diagnosis EF often based on association characteristic or subcutaneous abnormalities thickened fascia an inflammatory infiltration, mostly composed lymphocytes eosinophils. A peripheral eosinophilia frequently present (60-90%) but not mandatory for diagnosis. At onset, morphological might be helped muscle magnetic resonance imaging, which typically may evidence increased signal intensity within marked enhancement after gadolinium administration at acute phase disease. Differential diagnoses include eosinophilia-myalgia syndrome L-tryprophane ingestion, hypereosinophilic syndromes (HES), systemic sclerosis, eosinophilic granulomatosis polyangeitis, T cell lymphomas cutaneous involvement. There no consensual therapeutic strategy. However, oral corticosteroids, without methylprednisolone pluses, remain mainstay treatment significant improvement majority patients. It associated to immunosuppressive drug, mainly methotrexate, in patients morphea-like lesions unsatisfactory response corticosteroids alone.