Eosinophilic Fasciitis 30 Years after – What Do We Really Know?
Eosinophilic Fasciitis 30 Years after – What Do We Really Know?
复制标题
30 年后的嗜酸性筋膜炎——我们真正了解什么？
作者：
M. Antić;S. Lautenschlager;P. Itin
背景：嗜酸性筋膜炎是一种罕见的纤维性疾病，伴有外周嗜酸性粒细胞增多症和远端肢体硬皮病样硬化，严重影响患者的生活质量。虽然这种疾病早在30年前就已被描述，但其病因和发病机制仍不清楚，治疗上也缺乏共识。有大量的EF患者的病例报道，但系列报道很少。患者和方法：来自瑞士苏黎世Triemli医院皮肤科、巴塞尔大学医院皮肤科和Triemli医院皮肤科的11名EF患者进行了回顾性研究。结果：4例患者的初始诊断不被推荐的非皮肤科医生认可。中位年龄为55岁，不包括有史以来确诊为EF的最年轻患者(年龄=1岁)。所有患者都表现为皮肤硬化，其中3例导致关节疼痛痉挛。除2例患者外，其余患者均表现为水肿。上肢略占优势。硬指症1例。三名患者报告说，最初的创伤是在受影响的地方。两名患者的螺旋体病检测呈阳性。一名患者随后发展为再生障碍性贫血和桥本甲状腺炎。无内脏或皮外受累。8例患者在糖皮质激素治疗下完全或部分恢复，2例仅用环孢素、硫唑嘌呤或环磷酰胺治疗才能改善。结论：临床、实验室和组织学检查均可明确诊断。总体而言，皮质类固醇对EF非常有效，只有一小部分患者需要其他免疫抑制或细胞抑制药物。
Background: Eosinophilic fasciitis (EF) is a rare fibrosing disorder associated with peripheral eosinophilia and scleroderma-like induration of the distal extremities which affects substantially quality of life. Although the disease has been described 30 years ago, the etiology and pathomechanisms are still obscure, and consensus for therapy is lacking. Numerous case reports of patients with EF exist but series are scarce. Patients and Methods: Eleven patients with EF from the Department of Dermatology, Kantonsspital Aarau, the University Hospital Basel and the Outpatient Clinic of Dermatology, Triemli Hospital Zurich, Switzerland, were retrospectively studied. Results: In 4 patients the initial diagnosis was not recognized by the referring nondermatologists. The median age was 55 years, excluding the youngest patient ever diagnosed with EF (age = 1 year). All patients showed an induration of the skin, which led to painful contractures in the joints in 3 cases. All but 2 patients demonstrated edema. A slight predominance of the upper extremities was observed. Sclerodactyly was noticed in 1 patient. Three patients reported an initial trauma at the affected site. Two patients were tested positive for borreliosis. One patient subsequently developed aplastic anemia and Hashimoto thyroiditis. Visceral or extracutaneous involvement was absent. Eight patients had a full or partial recovery under corticosteroids whereas in 2, improvement could be achieved only with cyclosporine, azathioprine or cyclophosphamide. Conclusions: The diagnosis of EF can be established by clinical, laboratory and histological findings. In general, corticosteroids are highly efficacious in EF and only a minority of patients need other immunosuppressive or cytostatic drugs.
DOI：
10.1016/s0190-9622(03)00447-x
发表时间：
2003-12-01
影响因子：
13.8
作者：
French, LE;Shapiro, M;Rook, AH
通讯作者：
Rook, AH
影响因子：
2.3
作者：
Shakoory, B;Fitzgerald, SM;Krishnaswamy, G
通讯作者：
Krishnaswamy, G