Falcão Sandra, Mourão Ana F, Ribeiro Célia, Pinto Teresa L, Mateus Margarida, Araújo Paula, Nero Patrícia, Pimentão J Bravo, Branco J Cunha
Serviço de Reumatologia, Centro Hospitalar de Lisboa Ocidental, EPE, Hospital Egas Moniz, Lisboa.
Acta Reumatol Port. 2009 Jan-Mar;34(1):120-6.
Eosinophilic fasciitis is a rare rheumatic condition characterized by inflammatory thickening of the skin and fascia, peripheral eosinophilia, elevated erythrocyte sedimentation rate and hypergammaglobulinemia. Internal organ involvement is uncommon. It is often difficult to diagnose eosinophilic fasciitis and its course may be variable. Glucocorticoids are most commonly used in the treatment but in many cases they are ineffective, requiring combined immunosuppressive treatment. Several cases of eosinophilic fasciitis and serious haematological disorders such as immune thrombocytopenia, Hodgkin's disease and aplastic anaemia have been described. The authors report an atypical severe case of eosinophilic fasciitis complicated by aplastic anaemia non responsive to treatment.
嗜酸性筋膜炎是一种罕见的风湿性疾病,其特征为皮肤和筋膜的炎症性增厚、外周血嗜酸性粒细胞增多、红细胞沉降率升高和高球蛋白血症。内脏器官受累并不常见。嗜酸性筋膜炎常常难以诊断,其病程可能多变。糖皮质激素是最常用的治疗药物,但在许多情况下无效,需要联合免疫抑制治疗。已有数例嗜酸性筋膜炎合并严重血液系统疾病的病例报道,如免疫性血小板减少症、霍奇金病和再生障碍性贫血。作者报告了1例非典型重症嗜酸性筋膜炎病例,该病例合并再生障碍性贫血,治疗无效。