Eosinophilic fasciitis

Long H, et al. Eosinophilic skin diseases: a comprehensive review. Clin Rev Allergy Immunol. 2015 Apr 16. [Epub ahead of print]

“EF is generally a benign disease, with an exception that a few patients are associated with malignancies [184]. Some patients experienced spontaneous improvement of EF, although this is extremely rare [187]. Most patients are highly responsive to corticosteroid treatment, but quite a proportion of patients would still progress to incomplete remission with residual cutaneous fibrosis even after standard treatment. Several factors have been suggested to be associated with the prognosis, with regard to the outcome of residual fibrosis. Firstly, a young age (under 7 or 12 years old) at the time of onset, trunk involvement, and the occurrence of morphea-like skin lesions are found associated with a poor outcome with residual fibrosis [205]. On the other hand, earlier initiation of therapy [205] and the use of methylprednisolone pulses at initiation of treatment [186, 193] lead to a better outcome. Especially for patients with morphea-like skin lesions, the use of methotrexate as a second agent to combine with steroids is proposed for a better prognosis [186].”

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Pinal-Fernandez I, et al. Diagnosis and classification of eosinophilic fasciitis. Autoimmun Rev. 2014 Apr-May;13(4-5):379-82.

Eosinophilic fasciitis (EF) is a rare scleroderma-like syndrome with an unknown etiology and pathogenesis that should be considered an immune-allergic disorder. 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 affected skin showing inflammation and thickening of deep fascia is essential to establish the diagnosis. The differential diagnosis includes systemic sclerosis and other scleroderma subsets such as morphea, and epidemic fasciitis syndromes caused by toxic agents such as the myalgia-eosinophilia syndrome and toxic oil syndrome. Peripheral T cell lymphomas should also be ruled out. The diagnosis of EF can be established by clinical, laboratory and histological findings, but universally accepted international diagnostic criteria are lacking. Corticosteroids are efficacious and remain the standard therapy for EF, although some patients may improve spontaneously.

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Lebeaux D, Sène D. Eosinophilic fasciitis (Shulman disease). Best Pract Res Clin Rheumatol. 2012 Aug;26(4):449-58.

The 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, but is not mandatory for the EF diagnosis. The 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 should be ruled out, including eosinophilia-myalgia syndrome (EMS) after L-tryprophane ingestion, hypereosinophilic syndromes (HES), systemic sclerosis, Churg-Strauss syndrome, and/or peripheral T cell lymphomas with cutaneous involvement. Due to the scarcity of the EF disease, there is no consensual therapeutic strategy. However, oral corticosteroids remain the mainstay treatment and may be associated to an immunosuppressive drug such as methotrexate in patients with morphea-like lesions or an unsatisfactory response to corticosteroids alone.

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Hedrich CM, et al. Presentations and treatment of childhood scleroderma: localized scleroderma, eosinophilic fasciitis, systemic sclerosis, and graft-versus-host disease. Clin Pediatr (Phila). 2011 Jul;50(7):604-14.

“In the pediatric age group, EF is a rare connective tissue disease characterized by induration of the skin and soft tissues, peripheral eosinophilia, elevated levels of eosinophilic cationic protein, elevated ESR, and hypergammaglobulinemia. Clinical manifestations vary from adult cases, in that hands and ankles are usually affected in children. Disease course and response to therapy are in most cases comparable to adult cases. [4-11]”

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Loupasakis K, Derk CT. Eosinophilic fasciitis in a pediatric patient. J Clin Rheumatol. 2010 Apr;16(3):129-31.

We report the case of a pediatric patient with eosinophilic fasciitis, who was successfully treated with early high dose corticosteroids and subsequent use of mycophenolate mofetil. We believe that the early institution of corticosteroids helped to suppress the early inflammatory part of the disease and the subsequent use of mycophenolate mofetil maintained this and may have also helped prevent fibrotic skin changes.

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More PubMed results on eosinophilic fasciitis.

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