Department of Dermatology and Venereology, Istanbul University Faculty of Medicine, Istanbul, Turkey.
Department of Dermatology and Venereology, Istanbul University Faculty of Medicine, Istanbul, Turkey.
An Bras Dermatol. 2023 Nov-Dec;98(6):787-792. doi: 10.1016/j.abd.2022.09.012. Epub 2023 Jun 23.
Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are subtypes of pemphigus with distinct clinical and laboratory features. The transition between these two subtypes has rarely been reported previously.
The data of PV patients who exhibited clinical and immunoserological transition to PF during the follow-up period were retrospectively evaluated regarding their demographical, clinical, and laboratory characteristics.
Among 453 patients diagnosed with PV, 13 (2.9%) patients exhibited clinical and immunoserological transition from PV to PF. The mean age of PV patients at the time of diagnosis was 39.8 ± 14.7 (19‒62) years and 7 (53.8%) of them were female. These patients showed clinical and immunoserological transition from PV to PF after a period ranging from 4 months to 13 years (mean 36.2 ± 41 months). In addition to typical clinical features of PF, all patients had positive anti-desmoglein-1 and negative anti-desmoglein-3 antibody levels after the clinical transition had occurred without any mucosal involvement. During a mean 7.8 ± 5.8 (2‒21) years of follow-up period after the transition from PV to PF, only one female patient had experienced a re-transition to PV characterized by a relapse of disease involving mucosal surfaces with positive anti-desmoglein-3 antibody levels following a 5-year period of remission period without treatment.
Single-center study with a retrospective study design.
Our series is the largest group of patients reported to show the transition from PV to PF to date with a long follow-up period. The reason behind the disappearance of anti-desmoglein-3 antibodies and the pathogenesis of this phenomenon is not yet elucidated.
寻常型天疱疮（PV）和落叶型天疱疮（PF）是天疱疮的两种亚型，具有明显的临床和实验室特征。这两种亚型之间的转变以前很少有报道。
回顾性评估了在随访期间出现临床和免疫血清学向 PF 转变的 PV 患者的数据，评估其人口统计学、临床和实验室特征。
在 453 例诊断为 PV 的患者中，有 13 例（2.9%）患者出现了从 PV 向 PF 的临床和免疫血清学转变。PV 患者诊断时的平均年龄为 39.8 ± 14.7（19-62）岁，其中 7 例（53.8%）为女性。这些患者在 4 个月至 13 年（平均 36.2 ± 41 个月）的时间内出现从 PV 向 PF 的临床和免疫血清学转变。除了 PF 的典型临床特征外，所有患者在临床转变后均具有阳性抗桥粒芯糖蛋白 1 抗体和阴性抗桥粒芯糖蛋白 3 抗体水平，且无黏膜受累。在从 PV 向 PF 转变后的平均 7.8 ± 5.8（2-21）年的随访期间，仅 1 例女性患者经历了再向 PV 的转变，表现为疾病复发，累及黏膜表面，抗桥粒芯糖蛋白 3 抗体水平阳性，在未经治疗的 5 年缓解期后。
单中心研究，回顾性研究设计。
我们的系列是迄今为止报道的显示从 PV 向 PF 转变的最大患者群体，随访时间较长。抗桥粒芯糖蛋白 3 抗体消失的原因及其发病机制尚不清楚。
