Lévy-Sitbon C, Reguiaï Z, Durlach A, Goeldel A-L, Grange F, Bernard P
Service de dermatologie, hôpital Robert-Debré, CHU, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
Ann Dermatol Venereol. 2013 Dec;140(12):788-92. doi: 10.1016/j.annder.2013.07.013. Epub 2013 Aug 12.
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.
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.
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.
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。
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。
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。
