Eosinophilic fasciitis (Shulman's disease): Diagnostic and therapeutic review

Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by symmetrical and painful swelling with a progressive induration and thickening of the skin and soft tissues. The diagnosis of EF is often based on the association of characteristic skin or subcutaneous abnormalities and a thickened fascia with an inflammatory infiltration, mostly composed of lymphocytes and eosinophils. A peripheral eosinophilia is frequently present (60-90%) but is not mandatory for the EF diagnosis. At the onset, the morphological diagnosis might be helped by a muscle magnetic resonance imaging, which typically may evidence an increased signal intensity within the fascia and marked fascia enhancement after gadolinium administration at the acute phase of the disease. Differential diagnoses include eosinophilia-myalgia syndrome after L-tryprophane ingestion, hypereosinophilic syndromes (HES), systemic sclerosis, eosinophilic granulomatosis with polyangeitis, and peripheral T cell lymphomas with cutaneous involvement. There is no consensual therapeutic strategy. However, oral corticosteroids, with or without methylprednisolone pluses, remain the mainstay treatment with a significant improvement for the majority of patients. It might be associated to an immunosuppressive drug, mainly methotrexate, in patients with morphea-like lesions or an unsatisfactory response to corticosteroids alone. (C) 2015 Societe nationale francaise de medecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.