Bobrowska-Snarska Danuta, Ostanek Lidia, Brzosko Marek
Klinika Reumatologii Pomorskiej Akademii Medycznej w Szczecinie ul. Unii Lubelskiej 1, 71-252 Szczecin.
Ann Acad Med Stetin. 2008;54(2):47-51.
Eosinophilic fasciitis is a rare disease classified by some authors to scleroderma like syndromes. It occurs the most frequently between the second and the sixth decade of life, mainly in Caucasians. It usually appears in young males and exceptionally in children. It has an abrupt onset. The etiology of this disease is unknown. Hardening of the skin and subcutaneous tissue, eosinophilia in peripheral blood and hypergammaglobulinaemia are the most characteristic features of the disease. Inflammation and fibrosis of the fascia that spreads over into the deeper layers of skin and muscles are typical for its histological picture. The infiltration is composed of the lymphocytes, plasmocytes and eosinophiles. The involvement of internal organs in the course of the disease is rare. The course of eosinophilic fasciitis can be different. Prognosis is generally good, however recurrences of the disease can happen. The treatment with nonsteroidal anti-inflammatory drugs and/or with glucocorticosteroids is not satisfactory in some cases. It is not uncommon that intense immunosuppressive treatment is required.
嗜酸性筋膜炎是一种罕见疾病，一些作者将其归类为硬皮病样综合征。它最常发生在人生的第二个和第六个十年之间，主要发生在白种人中。它通常出现在年轻男性中，在儿童中极为罕见。其起病突然。这种疾病的病因尚不清楚。皮肤和皮下组织硬化、外周血嗜酸性粒细胞增多和高球蛋白血症是该疾病最具特征性的表现。蔓延至皮肤和肌肉深层的筋膜炎症和纤维化是其典型的组织学表现。浸润由淋巴细胞、浆细胞和嗜酸性粒细胞组成。在疾病过程中，内脏受累情况罕见。嗜酸性筋膜炎的病程可能不同。总体预后良好，然而疾病可能会复发。在某些情况下，使用非甾体抗炎药和/或糖皮质激素进行治疗并不令人满意。需要进行强化免疫抑制治疗的情况并不少见。
