嗜酸性筋膜炎

<正>嗜酸性筋膜炎(eosinophilic fasciitis,EF)是一种罕见的硬皮病样疾病,由Shulman~1]在1974年首次报道,以对称性的四肢肿痛、皮肤硬化、外周血嗜酸性粒细胞增多、高丙种球蛋白血症、血沉加快等为主要临床特征。EF在各年龄段均可发病,平均发病年龄多集中在40~50岁之间~2],男女发病率基本一致~3]。目前尚无国际诊断标准,EF的诊断多建立在典型的临

Eosinophilic fasciitis

Eosinophilic fasciitis (EF) is a rare scleroderma-like syndrome with an unknown etiology and pathogenesis.Peripheral blood eosinophilia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate are the main laboratory findings. Full-thickness wedge biopsy of the clinically affected skin showing in?ammation and thickening of deep fascia is essential to establish the diagnosis. Magnetic resonance imaging may evidence an increased signal intensity within the fascia.Differential diagnosis should be ruled out, including eosinophilia myalgia syndrome (EMS) after L-tryprophane ingestion, hypereosinophilic syndromes (HES), systemic sclerosis and Polymyositis. Oral corticosteroids remain the main-stay treatment and may be associated to an immunosuppressive drug and monoclonal antibody.