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[寻常型天疱疮转变为落叶型天疱疮:一例报告]

[Transition from pemphigus vulgaris to pemphigus foliaceus: a case report].

作者信息

Lévy-Sitbon C, Reguiaï Z, Durlach A, Goeldel A-L, Grange F, Bernard P

机构信息

Service de dermatologie, hôpital Robert-Debré, CHU, 45, rue Cognacq-Jay, 51092 Reims cedex, France.

出版信息

Ann Dermatol Venereol. 2013 Dec;140(12):788-92. doi: 10.1016/j.annder.2013.07.013. Epub 2013 Aug 12.

Abstract

BACKGROUND

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the two main subtypes of auto-immune pemphigus, each having different clinical, histological and immunopathological features. We report the case of a patient initially with typical PV who relapsed within 2years, presenting clinically, histologically and immunologically typical PF.

PATIENTS AND METHODS

A 47-year old man presented in March 2008 with clinically, histologically and serologically typical PV and treated with systemic corticosteroids alone (prednisone: 1mg/kg per day) then combined with a cycle of rituximab, which resulted in complete remission. After discontinuation of therapy (duration: 26months), he relapsed 6 months later with PF presenting clinical, histological and serological characteristics typical of this condition.

DISCUSSION

This is a rare case of complete transition from PV to PF in clinical, histological and serological terms, and the first case occurring after initial treatment with rituximab.

摘要

背景

寻常型天疱疮(PV)和落叶型天疱疮(PF)是自身免疫性天疱疮的两种主要亚型,各有不同的临床、组织学和免疫病理学特征。我们报告了一例最初表现为典型PV的患者,在2年内复发,临床、组织学和免疫学上呈现典型PF的病例。

患者与方法

一名47岁男性于2008年3月就诊,临床、组织学和血清学表现均为典型PV,仅接受全身皮质类固醇治疗(泼尼松:每日1mg/kg),随后联合一个疗程的利妥昔单抗治疗,实现完全缓解。治疗中断(持续时间:26个月)后,6个月后他复发为PF,呈现出该疾病典型的临床、组织学和血清学特征。

讨论

这是一例在临床、组织学和血清学方面从PV完全转变为PF的罕见病例,也是首例在最初使用利妥昔单抗治疗后出现这种情况的病例。

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