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血浆置换联合环磷酰胺成功治疗顽固性瘢痕性类天疱疮。

Successful treatment of recalcitrant cicatricial pemphigoid with a combination of plasma exchange and cyclophosphamide.

作者信息

Bohn J, Jonsson S, Holst R

机构信息

Departments of Dermatology and Haemoimmunotherapy, Vascular and Renal Diseases, Malmö University Hospital, S-205 02 Malmö, Sweden.

出版信息

Br J Dermatol. 1999 Sep;141(3):536-40. doi: 10.1046/j.1365-2133.1999.03054.x.

DOI:10.1046/j.1365-2133.1999.03054.x
PMID:10583063
Abstract

We describe two patients with severe oral cicatricial pemphigoid, one of whom also had severe pharyngeal involvement. Both patients were resistant to treatment with corticosteroids and other standard immunosuppressive therapies. Plasma exchange alone proved to be only temporarily effective, but the combination of plasma exchange with subsequent cyclophosphamide resulted in a remission in both patients. Both patients experienced mild side-effects during the plasma exchange treatment (urticaria and mild hypotension). At present, at follow-up of 6 and 9 years, respectively, the patients have no symptoms of active disease and have not required any further immunosuppressive treatment.

摘要

我们描述了两名患有严重口腔瘢痕性类天疱疮的患者,其中一名患者还伴有严重的咽部受累。两名患者对皮质类固醇和其他标准免疫抑制疗法均耐药。单独进行血浆置换仅证明有暂时疗效,但血浆置换联合随后的环磷酰胺治疗使两名患者均获缓解。两名患者在血浆置换治疗期间均出现了轻微副作用(荨麻疹和轻度低血压)。目前,分别在随访6年和9年时,这两名患者均无活动性疾病症状,也无需任何进一步的免疫抑制治疗。

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