Department of Dermatology and Allergology, University Hospital Marburg, Marburg, Germany.
Institute of Medical Bioinformatics and Biostatistics, Philipps-University Marburg, Marburg, Germany.
J Dtsch Dermatol Ges. 2022 Apr;20(4):461-467. doi: 10.1111/ddg.14695.
Pemphigus diseases are potentially life-threatening and rare autoimmune bullous disorders characterized by blisters and erosions of the skin and mucous membranes. These disorders can be largely divided into two major subtypes: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The objective of this study was to evaluate the autoantibody profile and response to therapy of PV and PF patients by analyzing the clinicopathological data from a registry for bullous autoimmune dermatoses.
In a retrospective study, data from 69 patients with PV and PF were included in the analysis. The Autoimmune Bullous Skin Intensity Score (ABSIS) was used to assess the clinical course, remissions, relapses and severity of the disease at first manifestation and throughout the observation period. ELISA was performed to assess levels of anti-desmoglein (Dsg)-1 and anti-Dsg3 IgG serum autoantibodies.
The mean remission time in PV and PF patients was 63 weeks. PV patients with mucosal involvement showed a more favorable healing process. In PV patients with a moderate/high anti-Dsg1 IgG serum level at baseline, anti-Dsg3 IgG levels decreased during the observation period.
Our study provides additional insights into the clinical course of patients with PV and PF, revealing that a mucosal phenotype is associated with a higher tendency towards remission.
天疱疮病是一种潜在危及生命的罕见自身免疫性大疱性疾病，其特征为皮肤和黏膜水疱和糜烂。这些疾病可大致分为两大主要亚型：寻常型天疱疮（PV）和落叶型天疱疮（PF）。本研究旨在通过分析大疱性自身免疫性皮肤病登记处的临床病理数据，评估 PV 和 PF 患者的自身抗体谱和治疗反应。
在一项回顾性研究中，纳入了 69 例 PV 和 PF 患者的数据。采用自身免疫性大疱皮肤严重程度评分（ABSIS）评估疾病首次表现和整个观察期的临床病程、缓解、复发和严重程度。通过 ELISA 评估抗桥粒芯糖蛋白 1（Dsg）-1 和抗 Dsg3 IgG 血清自身抗体水平。
PV 和 PF 患者的平均缓解时间为 63 周。黏膜受累的 PV 患者具有更有利的愈合过程。在基线时具有中/高抗 Dsg1 IgG 血清水平的 PV 患者中，抗 Dsg3 IgG 水平在观察期间下降。
本研究为 PV 和 PF 患者的临床病程提供了更多的见解，表明黏膜表型与更高的缓解倾向相关。
