Diagnostic criteria, severity classification and guidelines of eosinophilic fasciitis

Abstract

We established diagnostic criteria and severity classification of eosinophilic fasciitis because there is no established diagnostic criteria or widely accepted severity classification of the disease. Also, there has been no clinical guideline for eosinophilic fasciitis, so we established its clinical guideline ahead of all over the world. In particular, the clinical guideline was established by clinical questions based on evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guideline easy to use and reliable based on the newest evidence, and to present guidance as specific as possible for various clinical problems in treatment of eosinophilic fasciitis.

Eosinophilic Fasciitis of Wrists with a Positive Prayer Sign

“…In regard to therapy, data from retrospective studies increasingly favor the combination of systemic corticosteroids (prednisone 0.5-1 mg/kg/day) and methotrexate (10-25 mg once weekly) as the initial treatment [14]. A study showed that patients had a response rate of more than 70% to oral prednisone [15].…”

Steroid‐resistant eosinophilic fasciitis successfully treated with addition of extracorporeal photopheresis

“…To date, various treatments have been tried in steroid‐resistant cases, most of them with anecdotal evidence to support their use (methotrexate being an exception). These include mycophenolate mofetil, cyclosporin, azathioprine, cyclophosphamide, dapsone, ketotifen, cimetidine, infliximab, and antimalarials (Fett & Arthur, ; Jinnin et al, ; Mazori et al, ).…”

“…Women are 1.3-2.1 times as likely as men to be affected (Mazori et al, 2017). A previous history of intense physical exertion or trauma has classically been considered as a triggering event (Fett & Arthur, 2018;Romano et al, 2003), but more recent studies suggest that physical stress may be less of a risk factor than previously thought (Jinnin et al, 2018;Mazori et al, 2017).…”

“…(b) November 2018: considerable improvement in cobblestone appearance and underlying induration, with persistence of mild hyperpigmentation but in a smaller area F I G U R E 2 Biopsy specimen obtained from the thigh revealing thickening of the deep fascia with a predominantly lymphocytic infiltrate with some eosinophils. Lymphocytes on the surrounding fat can also be seen (hematoxylin-eosin, original magnification ×40) azathioprine, cyclophosphamide, dapsone, ketotifen, cimetidine, infliximab, and antimalarials (Fett & Arthur, 2018;Jinnin et al, 2018;Mazori et al, 2017).…”

Eosinophilic fasciitis associated with myositis: Report of four cases and review of the literature

“…Eosinophilic fasciitis, or Shulman syndrome, is a rare scleroderma‐like disorder that was first described in 1975 as a variant of scleroderma with eosinophilia and fasciitis; it is characterised by symmetrical and painful swelling, progressive induration, thickening of the skin, peripheral eosinophilia, hypergammaglobulinemia and an elevated erythrocyte sedimentation rate (ESR) . Eosinophilic myositis is characterised by lymphoplasmacytic muscle infiltration, which is rarely observed.…”