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[嗜酸性筋膜炎(舒尔曼综合征)作为硬皮病样皮肤改变的一种罕见鉴别诊断]

[Eosinophilic fasciitis (Shulman syndrome) as a rare differential diagnosis of sclerodermiform skin changes].

作者信息

Hamm M, Rubart M, Behrenbeck H P, Röttger P, Leonhardt H

机构信息

Medizinische Klinik II des Krankenhauses Düren.

出版信息

Dtsch Med Wochenschr. 1993 Nov 26;118(47):1722-6. doi: 10.1055/s-2008-1059508.

Abstract

For 3 months, a 70-year-old woman had been affected by painless, symmetrical swellings in the limbs, spreading proximally, causing hardening of the skin and impaired joint movements, but no fever. Her general condition and findings on physical examination were according to age. ESR was 22/42 mm and there was a thrombocytosis (533,000/microliters) and eosinophilia (16%, absolute 2,140/microliters). Radiological and ultrasound examinations were normal. Skin biopsy revealed marked oedema of the connective tissue septa and perivascular lymphohistiocytic infiltrations, which--together with the other findings--suggested eosinophilic fasciitis. She was treated with prednisone, 100 mg daily, reduced gradually to 8 mg daily, as well as cimetidine, 800 mg daily, as immunomodulator, and intensive physiotherapy. The swellings and eosinophilia regressed within 8 weeks, but the hardening and impaired mobility persisted. Maintenance glucocorticoids are being continued.

摘要

三个月来,一名70岁女性四肢出现无痛性对称性肿胀,向近端蔓延,导致皮肤硬化和关节活动受限,但无发热。她的一般状况和体格检查结果与年龄相符。血沉为22/42毫米,存在血小板增多症(533,000/微升)和嗜酸性粒细胞增多(16%,绝对值2,140/微升)。放射学和超声检查正常。皮肤活检显示结缔组织间隔明显水肿和血管周围淋巴细胞及组织细胞浸润,结合其他发现提示嗜酸性筋膜炎。她接受了泼尼松治疗,每日100毫克,逐渐减至每日8毫克,同时服用西咪替丁,每日800毫克作为免疫调节剂,并接受强化物理治疗。肿胀和嗜酸性粒细胞增多在8周内消退,但皮肤硬化和活动受限持续存在。目前仍在继续使用维持剂量的糖皮质激素。

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