Search results for "Eosinophilic fasciitis"
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205th ENMC International Workshop: Pathology diagnosis of idiopathic inflammatory myopathies Part II 28-30 March 2014, Naarden, The Netherlands.
2015
The idiopathic inflammatory myopathies (IM) are a heterogeneous group of diseases and diagnosis often necessitates a muscle biopsy. Five main entities are recognized: (1) dermatomyositis (DM); (2) polymyositis (PM); (3) necrotizing autoimmune myopathy (NAM); (4) sporadic inclusion body myositis (IBM); and (5) non-specific myositis. Other entities include granulomatous myopathy, macrophagic myofasciitis, and eosinophilic fasciitis (Shulman's syndrome). The pathological classification and subsequent identification of disease subgroups are extremely important for assessing treatment options and prognosis in the individual patient, yet classification criteria have not been standardized and vali…
P.21.2 New insights into eosinophilic fasciitis
2013
Eosinophilic fasciitis (EF), first described by Shulman in 1974, is a rare disease characterized by fibrosis and inflammatory infiltration of the muscle fascia as well as scleroderma-like skin indurations and blood eosinophilia. In contrast to other inflammatory myopathies, patients generally show less muscle weakness and myalgia, and a frequent increase in body weight. Thus, we hypothesize a unique immune mechanism underlying Shulman syndrome. The immunohistochemical expression pattern of leucocytes and a comprehensive panel of cytokine and chemokine expression on RNA level of muscle specimen from EF patients were compared to healthy control muscle. In patients with biopsy-proven EF the im…
Steroid-resistant eosinophilic fasciitis successfully treated with addition of extracorporeal photopheresis.
2019
Eosinophilic fasciitis (EF) is an uncommon fibrosing disease of the fascia with characteristic cutaneous and hematologic manifestations. Although EF is most commonly treated with corticosteroids at the beginning, a considerable number of patients show an inadequate response and hence various therapeutic strategies have been tried, including extracorporeal photopheresis (ECP). We describe the case of a 66-year-old woman with steroid-resistant EF that improved significantly after ECP was added to her treatment regimen. To date, only six cases of this therapeutic strategy have been reported in English literature.