Welc Natalia, Ważniewicz Sandra, Głuszak Paweł, Spałek Maciej, Seraszek-Jaros Agnieszka, Jałowska Magdalena, Dmochowski Marian
Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, 61701 Poznan, Poland.
Department of Bioinformatics and Computational Biology, Poznan University of Medical Sciences, 61701 Poznan, Poland.
Antibodies (Basel). 2024 Dec 13;13(4):103. doi: 10.3390/antib13040103.
BACKGROUND/OBJECTIVES: Pemphigus comprises a diverse group of disorders within the autoimmune bullous dermatoses (AIBDs) spectrum. Among these, pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the most commonly encountered variants. Despite its rarity, this condition can pose a life-threatening risk. We aimed to assess clinical characteristics, comorbidities, medication, as well as the treatment of various types of pemphigus in pemphigus patients.
We gathered data from 69 patients treated in the Department of Dermatology in the years 2016-2023. The investigation included sex, age at diagnosis, type of pemphigus, comorbidities and medications, presence of neoplasms and treatment of pemphigus, as well as enzyme-linked immunosorbent assay (ELISA) and direct immunofluorescence (DIF) results. The data were statistically analyzed with the -value set at 0.05.
The study group comprised 69 patients, including 41 women and 28 men. The mean age at diagnosis was 56.89 years +/- 15.42 years. A total of 79.31% of the patients were diagnosed with PV and the following 26.09% with PF. The most common comorbidities were arterial hypertension, hypercholesterolemia, and diabetes mellitus. The dominant treatment regimen was the systemic use of glucocorticosteroids (GCSs; 90% and 94% of PV and PF patients, respectively). More than half of the patients received at least one GCS-sparing treatment, including dapsone and rituximab. We observed a significantly frequent presence of IgG deposits in DIF in patients with PF ( = 0.0217) and a subsequent correlation between the concurrent presence of IgG deposits in DIF and anti-DSG1 antibodies in ELISA testing ( = 0.0469). The combination of IgG, IgG1, IgG4, and C3 deposits was more often existent in PF patients ( = 0.0054) and the combination of IgG4 and C3 deposits in PV patients ( = 0.0339). We also found a positive correlation between the level of anti-DSG1 antibodies and the age at diagnosis ( = 0.0298).
Patients with pemphigus are very often diagnosed with significant comorbidities and take diverse medication, which shows that the treatment of pemphigus should follow a multidisciplinary approach. Accurate analysis of the clinical condition of the patients, as well as the results of the ELISA panel or DIF, is crucial for a successful diagnostic and therapeutic process.
背景/目的：天疱疮是自身免疫性大疱性皮肤病（AIBDs）谱系中的一组多种疾病。其中，寻常型天疱疮（PV）和落叶型天疱疮（PF）是最常见的类型。尽管这种疾病罕见，但可能构成危及生命的风险。我们旨在评估天疱疮患者中各种类型天疱疮的临床特征、合并症、用药情况以及治疗方法。
我们收集了2016年至2023年在皮肤科接受治疗的69例患者的数据。调查内容包括性别、诊断时年龄、天疱疮类型、合并症和用药情况、肿瘤的存在及天疱疮的治疗，以及酶联免疫吸附测定（ELISA）和直接免疫荧光（DIF）结果。数据采用统计学分析，设定P值为0.05。
研究组包括69例患者，其中41例女性和28例男性。诊断时的平均年龄为56.89岁±15.42岁。共有79.31%的患者被诊断为PV，接下来26.09%的患者被诊断为PF。最常见的合并症是动脉高血压、高胆固醇血症和糖尿病。主要治疗方案是全身使用糖皮质激素（GCSs；PV和PF患者分别为90%和94%）。超过一半的患者接受了至少一种糖皮质激素节省治疗，包括氨苯砜和利妥昔单抗。我们观察到PF患者在DIF中IgG沉积明显更频繁（P = 0.0217），并且在ELISA检测中DIF中IgG沉积与抗DSG1抗体同时存在之间存在相关性（P = 0.0469）。IgG、IgG1、IgG4和C3沉积的组合在PF患者中更常出现（P = 0.0054），而IgG4和C3沉积的组合在PV患者中更常出现（P = 0.0339）。我们还发现抗DSG1抗体水平与诊断时年龄之间存在正相关（P = 0.0298）。
天疱疮患者常被诊断出有严重的合并症并服用多种药物，这表明天疱疮的治疗应遵循多学科方法。准确分析患者的临床状况以及ELISA检测或DIF结果对于成功的诊断和治疗过程至关重要。
