Rare Skin Diseases Unit, Section of Dermatology, Department of Health Sciences, Unità Sanitaria Locale (USL) Toscana Centro, European Reference Network-Skin Member, University of Florence, Florence, Italy.
Unit of Epidemiology of Rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy.
Front Immunol. 2022 Jul 8;13:895490. doi: 10.3389/fimmu.2022.895490. eCollection 2022.
Pemphigus encompasses a group of muco-cutaneous autoimmune bullous diseases characterized by the loss of adhesion between keratinocytes. The disease is associated with increased morbidity and mortality.
We characterized clinical patterns, survival, comorbidities, and drug prescriptions in patients with pemphigus referred to the Section of Dermatology of the University of Florence from January 2010 to December 2021.
A total of 149 patients were identified (female/male sex ratio = 2.0). Median age at diagnosis was 57.7 ± 17.2 years; 108 patients were diagnosed with pemphigus vulgaris (PV) (72.5%) and 35 (23.5%) with pemphigus foliaceus (PF). Paraneoplastic pemphigus (PNP) and IgA-pemphigus accounted for three patients each. The overall survival rate was 86.9%. Accordingly, 14 (9%) patients died during the study period. The average age at death was 77.8 ± 9.3. Age at diagnosis was a risk factor for death in patients with pemphigus. Average concentration of Dsg3-IgG and Dsg1-IgG was 85.6 ± 68.8 and 75.9 ± 68.4, respectively. The most serious comorbid diseases included cerebro- and cardiovascular accidents and malignancies. Regarding the treatment regimen, we found a substantially stable use of systemic steroids in the 2010-2018 period; the prevalence of use of mycophenolic acid increased, whereas that of azathioprine decreased. The use of rituximab showed the highest increase in the 2013-2018 period. Proton-pump inhibitors and antibiotics were the most frequently prescribed non-immunomodulating drugs.
In this large series of the patients, patients with pemphigus showed a high incidence of serious comorbid diseases, highlighting the importance of a multidisciplinary approach for a proper management of the patients. Rituximab was the immunomodulating drug showing the highest increase in use over time, reflecting the growing evidence of its efficacy as a first-line treatment in pemphigus.
天疱疮是一组以角质形成细胞间黏附丧失为特征的黏膜皮肤自身免疫性大疱性疾病。该疾病与较高的发病率和死亡率相关。
我们对 2010 年 1 月至 2021 年 12 月期间佛罗伦萨大学皮肤科收治的天疱疮患者的临床模式、生存情况、合并症和药物处方进行了特征描述。
共鉴定出 149 名患者（男女比例为 2.0）。诊断时的中位年龄为 57.7 ± 17.2 岁；108 名患者诊断为寻常型天疱疮（PV）（72.5%），35 名（23.5%）诊断为落叶型天疱疮（PF）。副肿瘤性天疱疮（PNP）和 IgA 天疱疮各占 3 例。总体生存率为 86.9%。因此，在研究期间，有 14 名（9%）患者死亡。死亡患者的平均年龄为 77.8 ± 9.3 岁。年龄是天疱疮患者死亡的危险因素。天疱疮患者的平均 Dsg3-IgG 和 Dsg1-IgG 浓度分别为 85.6 ± 68.8 和 75.9 ± 68.4。最严重的合并症包括脑和心血管疾病和恶性肿瘤。关于治疗方案，我们发现 2010-2018 年期间全身类固醇的使用基本稳定；霉酚酸的使用率增加，而硫唑嘌呤的使用率降低。利妥昔单抗的使用在 2013-2018 年期间呈最高增长。质子泵抑制剂和抗生素是最常开的非免疫调节药物。
在这项大型患者系列研究中，天疱疮患者的严重合并症发生率较高，突出了多学科方法对患者进行适当管理的重要性。利妥昔单抗是免疫调节药物，随着时间的推移，其使用量呈最高增长，反映了其作为天疱疮一线治疗药物的疗效证据不断增加。
